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“This excellent book is, in equal parts, fascinating and instructive! So much
of our neuropsychological knowledge has emerged from the study of rare
conditions and this book continues this vital tradition. By developing a deep
understanding of a wide range of rare conditions, diagnostic challenges and
controversial issues, we also improve our knowledge of how to manage con-
ditions that are more common in clinical practice. I really enjoyed reading
this book and thoroughly recommend it!”
Jon Evans, Professor of Clinical Neuropsychology,
University of Glasgow

“This exceptional text is both groundbreaking and instructive. The detailed

and clear presentation of rare cases accompanied by assessment findings, treat-
ment protocols, theoretical implications and patient perspectives provides a
roadmap for how to approach complex cases. The work of every clinician
and researcher working to enhance the lives of individuals with challenging
neurological conditions will be positively impacted by reading this volume
and applying its concepts.”
McKay Moore Sohlberg, PhD, University of Oregon

“An important text on the complexities of diagnosis in neuropsychology with

fascinating case histories by leaders in the field.”
Ian Robertson, Global Brain Health Institute,
Trinity College Dublin
Rare Conditions, Diagnostic
Challenges, and Controversies in
Clinical Neuropsychology

This book highlights those rare, difficult to diagnose, or controversial cases

in contemporary clinical neuropsychology. The evidence base relevant to
this type of work is almost by definition insufficient to guide practice, but
most clinicians will encounter such cases at some point in their careers. By
documenting the experiences and learning of clinicians who have worked
with cases that are ‘out of the ordinary’, the book addresses an important gap
in the literature.
The book discusses 23 challenging and fascinating cases that fall outside
what can be considered routine practice. Divided into three sections, the text
begins by addressing rare and unusual conditions, defined as either conditions
with a low incidence, or cases with an atypical presentation of a condition. It
goes on to examine circumstances where an accurate diagnosis and/or coher-
ent case formulation has been difficult to reach. The final section addresses
controversial conditions in neuropsychology, including those where there is
ongoing scientific debate, disagreement between important stakeholders, or
an associated high-stakes decision. This text covers practice across lifespan
and offers crucial information on specific conditions as well as implications
for practice in rare disorders.
This book will be beneficial for clinical neuropsychologists and applied
psychologists working with people with complex neurological conditions,
along with individuals from medical, nursing, allied health, and social work
backgrounds. It will further be of appeal to educators, researchers, and stu-
dents of these professions and disciplines.

Dr Jessica Fish is a clinical psychologist and neuropsychologist. Trained at

the universities of Exeter, Cambridge, and King’s College London, she is a
lecturer and researcher at the University of Glasgow, and works clinically at
St George’s Hospital, London. Her primary expertise is in acquired brain
injury and neuropsychological rehabilitation.

Dr Shai Betteridge is Consultant Clinical Neuropsychologist and Chief

Psychological Professions Officer at St George’s University Hospitals NHS
Foundation Trust, and a founder and director of Allied Neuro Therapy Ltd.
Her fields of expertise include neuropsychological rehabilitation, service
development, quality improvement, and clinical excellence, spanning both
public and private sectors.

Dr Barbara A. Wilson is a world-renowned clinical neuropsychologist.

Now retired, Barbara has developed eight neuropsychological tests, writ-
ten 32 books, and published more than 300 papers and chapters. Her main
contributions are in ecologically valid assessment approaches, cognitive re-
habilitation and errorless learning, the holistic model of rehabilitation, and
disorders of consciousness.
Rare Conditions, Diagnostic
Challenges, and Controversies
in Clinical Neuropsychology
Out of the Ordinary

Edited by
Jessica Fish, Shai Betteridge,
and Barbara A. Wilson
Designed cover image: Getty Images
First published 2023
by Routledge
4 Park Square, Milton Park, Abingdon, Oxon OX14 4RN
and by Routledge
605 Third Avenue, New York, NY 10158
Routledge is an imprint of the Taylor & Francis Group, an informa business
© 2023 selection and editorial matter, Jessica Fish, Shai Betteridge,
and Barbara A. Wilson; individual chapters, the contributors
The right of Jessica Fish, Shai Betteridge, and Barbara A. Wilson
to be identified as the authors of the editorial material, and of the
authors for their individual chapters, has been asserted in accordance
with sections 77 and 78 of the Copyright, Designs and Patents Act
1988.
All rights reserved. No part of this book may be reprinted or
reproduced or utilised in any form or by any electronic, mechanical,
or other means, now known or hereafter invented, including
photocopying and recording, or in any information storage or
retrieval system, without permission in writing from the publishers.
Trademark notice: Product or corporate names may be trademarks
or registered trademarks, and are used only for identification and
explanation without intent to infringe.
British Library Cataloguing-in-Publication Data
A catalogue record for this book is available from the British Library

ISBN: 9781032132259 (hbk)

ISBN: 9781032132242 (pbk)
ISBN: 9781003228226 (ebk)

DOI: 10.4324/9781003228226
Typeset in Bembo
by codeMantra
This book is dedicated to the people featured in its
chapters as patients or clients, along with their families.
We recognise that this book represents a perspective on
some of the most difficult life experiences and we thank
you for allowing us to learn from you and to share this
learning in the hope that we can help others in future.
Contents

List of contributors xiii

Acknowledgements xvii

1 Introduction: rare conditions, diagnostic challenges,

and controversies in clinical neuropsychology 1
J ESSICA FISH, SH A I BET T ER I DGE , A N D BA R BA R A A. W I L SON

PART 1
Rare and unusual conditions 7

2 Gas geyser syndrome in India: a tragic, preventable

neuropsychological morbidity 9
U RVA S H I S H A H

3 Disconnection syndrome and optic aphasia following

left hemisphere posterior cerebral artery stroke:
a deductive assessment approach 24
J OA N N A AT K I N S O N

4 Creutzfeldt-Jakob disease presenting with

Capgras syndrome 41
NA R IN DER K A PU R A N D NOR M A N POOLE

5 A rare and challenging differential diagnosis:

prosopagnosia and reduced empathy in right-variant
semantic dementia – where ‘understanding does not map
onto reality’ 51
J U LI A COOK
x Contents
6 A life in Portrait mode: living with Balint’s syndrome 73
J WA L A N A R AYA N A N

7 Exploring the unknown: shared discovery in rare

mitochondrial disease 86
BEN M A R R A M

8 Galactosaemia: a rare metabolic disorder associated

with ‘hidden’ deficits and social vulnerability 101
S T E P H A N I E S ATA R I A N O, L O U I S E E DWA R D S , A N D RO S H N I VA R A

9 Anti-N-methyl-D-aspartate receptor antibody

encephalitis: post-acute neuropsychological
consequences and rehabilitation in adolescence 110
C AT H E R I N E H A RT E R A N D F E RG U S G R AC E Y

PART 2
Diagnostic challenges 131

10 Neuropsychological, neuropsychiatric, and functional

neurological symptoms: the challenges of overlapping
and evolving presentations 133
A L E X A N D R A E . RO S E A N D M I C H A E L D I L L E Y

11 Factitious disorder after severe head injury 149

A N D R E W WO RT H I N G T O N

12 Deafness or brain injury? diagnostic overshadowing in

a deaf person with bilateral temporal lobe damage and
visual agnosia 162
J OA N N A AT K I N S O N A N D DA R R E N T OW N S E N D - H A N D S C O M E

13 Aerotoxic syndrome: are passengers and aircrew

breathing toxic cabin air? 191
S A R A H M AC K E N Z I E RO S S

14 Focal anterograde amnesia: an extraordinary case 206

G E O RG I N A B ROW N E
Contents xi
xii Contents
Contributors

Dr Karen Addy, Salomons Institute for Applied Psychology, Canterbury

Christ Church University, Tunbridge Wells. karen.addy@canterbury.
ac.uk
Dr Joanna Atkinson, Deafness, Cognition and Language Research Centre,
University College London. joanna.atkinson@ucl.ac.uk
Dr Shai Betteridge, Clinical Neuropsychology & Clinical Health Psychol-
ogy, St George’s University Hospitals NHS Foundation Trust, London;
Allied Neuro Therapy, Egham, Surrey. shai.betteridge@stgeorges.nhs.uk
Dr Georgina Browne, Neuropsychology Department, Addenbrooke’s
Hospital, Cambridge. georgina.browne@addenbrookes.nhs.uk
Dr Laura Carroll, The Children’s Trust, Tadworth.
Dr Enrique Childress, The Children’s Trust, Tadworth.
Professor Rudi Coetzer, The Disabilities Trust, Silkwood Park, Wakefield;
School of Human & Behavioural Sciences, Bangor University, Wales;
Faulty of Medicine, Health & Life Science, Swansea University, Wales.
Email: b.r.coetzer@bangor.ac.uk.
Dr Sal Connolly, The Royal Hospital for Neuro-disability, Putney, Lon-
don; Connolly Neuro, Harley Street, London.
Dr Sarah Crawford, The Royal Hospital for Neuro-disability, Putney,
London. scrawford@rhn.org.uk
Dr Michael Dilley, Brain & Mind Ltd, London; Kings College Hospital
NHS Foundation Trust, London. Michael.Dilley@nhs.net
Louise Edwards, Independent Speech & Language Therapist, London.
Dr Sally Finnie, Department of Neuropsychology, Reading. Sally.Finnie@
berkshire.nhs.uk
Dr Jessica Fish, School of Health & Wellbeing, University of Glasgow,
Glasgow, UK; Clinical Neuropsychology & Clinical Health Psychology,
St George’s University Hospitals NHS Foundation Trust, London. jessica.
fish@glasgow.ac.uk
xiv Contributors
Dr Fergus Gracey, Department of Clinical Psychology and Psychological
Therapy, Norwich Medical School, University of East Anglia, Norwich.
f.gracey@uea.ac.uk
Dr Andrew Hanrahan, The Royal Hospital for Neuro-disability, Putney,
London.
Dr Catherine Harter, The Cambridge Centre for Paediatric Neuropsy-
chological Rehabilitation (CCPNR), Cambridge and Peterborough NHS
Foundation Trust. catherine.harter@nhs.net
Dr Jonathan Hinchliffe, The Royal Hospital for Neuro-disability, Putney,
London; Cognisant Neuropsychology Ltd.
Dr Jenny Jim, The Children’s Trust, Tadworth; University College Lon-
don. jjim@thechildrenstrust.org.uk
Prof. Narinder Kapur, University College London; Imperial College
NHS Trust, London. n.kapur@ucl.ac.uk
Dr Leigh Leppard, Lishman Neuropsychiatry Unit, South London and
Maudsley NHS Trust, London. leigh@leppardpsychology.co.uk
Dr Valeria Lowing, The Children’s Trust, Tadworth.
Prof. Sarah Mackenzie Ross, Research Department of Clinical, Edu-
cational & Health Psychology, University College London. s.macken-
zie-ross@ucl.ac.uk
Dr Paolo Mantovani
Dr Ben Marram, Community Neurological Rehabilitation Service, Leeds
Community Healthcare NHS Trust. benjamin.marram@nhs.net
Jwala Narayanan, Department of Neurology, Manipal Hospitals & Depart-
ment of Neuropsychology, Annasawmy Mudaliar General Hospital. jwala.
narayanan@gmail.com
Dr Elena Olgiati, The Royal Hospital for Neuro-disability, Putney, Lon-
don; Imperial College London, Department of Brain Sciences.
Dr Louise Owen, The Children’s Trust, Tadworth.
Dr Norman Poole, Department of Neuropsychiatry, St George’s Hospital,
South West London and St George’s Mental Health NHS Trust.
Dr Priyanka Pradhan, St George’s University Hospitals NHS Foundation
Trust, London.
Dr Elizabeth Roberts, The Children’s Trust, Tadworth.
Ms Alexandra E. Rose, Royal Hospital for Neuro-Disability, London;
Mental Health & Wellbeing, School of Health & Wellbeing, University of
Glasgow. arose@rhn.org.uk
 Contributors xv
Dr Stephanie Satariano, Evelina Children’s Hospital, London; Child Psy-
chology, London. stephanie@childpsychology.london
Dr Urvashi Shah, Department of Neurology, King Edward Memorial
Hospital Mumbai, India. shahurvashi100@gmail.com
Dr Isabelle Sharples, The Children’s Trust, Tadworth.
Dr Sonja Soeterik, Neurolink Psychology, London. dr.soeterik@neu-
rolinkpsych.co.uk
Dr Victoria Teggart, Greater Manchester Mental Health NHS Foundation
Trust, Manchester. viki.teggart@gmmh.nhs.uk
Darren Townsend-Handscomb, London.
Dr Roshni Vara, Evelina Children’s Hospital, London.
Dr Barbara A. Wilson, Clinical Neuropsychology & Clinical Health Psy-
chology, St George’s University Hospitals NHS Foundation Trust, Lon-
don; Allied Neuro Therapy, Egham, Surrey. barbara.wilson00@gmail.
com
Prof. Andrew Worthington, Headwise, Birmingham; Faculty of Health,
Medicine and Life Science, Swansea University, Swansea. aworthington@
headwise.org.uk
Acknowledgements

We would first like to express our gratitude to the chapter authors for vol-
unteering to contribute to this volume and for writing about their clinical
work. Many of the chapters relate to complex and non-routine aspects of
clinical practice which may have felt like a professional ‘stretch’, and that
may not have reached a satisfactory conclusion. To conduct this work takes
a great deal of creativity and resourcefulness as well as essential knowledge
and experience. Writing about this work takes valuable time, effort, and per-
haps the odd wrangle with one’s inner critic. Sharing this work with others
takes bravery and demonstrates a commitment to learning and development
as individuals and beyond for our services and professional disciplines. Sev-
eral authors demonstrated phenomenal patience when awaiting our feedback,
and nonetheless responded swiftly to queries and requests for amendments.
We thank you sincerely for all of this and hope that you are pleased with the
finished product.
We would also like to thank our colleagues at the University of Glasgow, St
George’s University Hospitals NHS Foundation Trust, Allied Neuro Therapy
Ltd, the Encephalitis Society, and the British Psychological Society Division
of Neuropsychology for their support; in particular: Hamish McLeod, Jon Ev-
ans, Katherine Carpenter, Ingram Wright, Alexandra Rose, Gaby Parker, Juliet
Lawson, and Tasneem Mohamed. We also acknowledge the late Mick Wilson
for his support at the beginning of this project. His encouragement to write
about our clinical neuropsychological practice will be an eternal inspiration to
all of us.
Finally, we would like to thank Lucy Kennedy, Lakshay Gaba and the
team at Routledge for their guidance, encouragement, insight, and patience
throughout the process of producing this book.
1 Introduction
Rare conditions, diagnostic
challenges, and controversies in
clinical neuropsychology
Jessica Fish, Shai Betteridge, and Barbara A. Wilson

We have an admittedly entirely biased perspective that clinical neuropsy-

chologists are ideally placed to work with people with rare disorders, to grap-
ple with difficult diagnostic questions, and to engage with controversial topics
within clinical neurosciences. The academic discipline of neuropsychology
is founded on the study of rare cases; people with focal brain injuries whose
specific cognitive impairments provided a wealth of information about the
structure of the human mind and spawning more box-and-arrow diagrams
than it would be feasible to count (for overviews see Marshall & Gurd, 2010;
Vallar & Caputi, 2020; and the canon – Shallice, 1988). In contrast, training
in clinical psychology provides a thorough grounding in the full range of
mental health conditions as well as other areas of specialism, alongside psy-
chological formulation and intervention from within a broad biopsychosocial
framework (see British Psychological Society (BPS), 2019a). These disciplines
converge in the profession of clinical neuropsychology, and the range of skills
clinical neuropsychologists possess (see BPS, 2019b) applies extremely well
to the care of people with complex neurological conditions. This is not to
say that clinical neuropsychology is the only nor indeed the most important
profession; the best care for people with complex neuro conditions undoubt-
edly arises from working in integrated systems and interdisciplinary teams
towards shared goals – many hands making light work (e.g. Bernard et al.,
2010; Wilson et al., 2009).
This book arose from discussions about various ‘difficult’ cases we and our
colleagues have encountered in clinical practice. It is very important that we
make clear from the outset that, when we refer to such cases, we do not mean
that the patients themselves are difficult, rather we mean that the circumstances of
the cases have been difficult. Many factors can contribute to such difficulties,
and we have grouped these into three broad categories as follows:

1. Rare and unusual conditions. Within this, we include:

a. Conditions that have a low incidence in the population, such as mi-

tochondrial disorders, rare metabolic syndromes or prion diseases,

DOI: 10.4324/9781003228226-1
2 Introduction
which outside the context of specialist services may be hard to rec-
ognise, poorly understood, and have inadequate provision; and
b. Cases with an atypical presentation of a condition, where the under-
lying condition may or may not be so rare. For example, in Chapter
3 a rare disconnection syndrome is found to result from a not-so-
rare left posterior cerebral artery stroke. In Chapter 4, a case of the
rare prion disease Creutzfeld-Jacob disease is identified presenting
with the also rather rare Capgras delusion; and the cases in Chapter
6 who presented with the rare neuropsychological condition Balint’s
syndrome as a result of rare neurological manifestations of the not-
so-rare systemic conditions dengue fever and Covid-19.

2. Cases where an accurate diagnosis and/or coherent case formu-

lation is difficult to reach. The rare and/or atypical conditions in the
first category may of course be difficult to formulate and diagnose, but
a range of other circumstances may also complicate assessment, formula-
tion, and diagnosis, for instance:

a. The presence of comorbid conditions that ‘muddy the waters’ of

what might otherwise be a clear-cut issue. The most striking exam-
ple of this is in chapter 12, where a person’s deafness overshadowed
the identification of a brain injury for a period of decades.
b. The limits of our current assessment tools. For example, where there
is no standardised/stand-alone battery for identifying the condition,
or if the tools that do exist are not accessible for reasons of disability,
culture etc. (as illustrated in many of our chapters).
c. Limits associated with the service provision. The commissioning ar-
rangements of some services mean that access to tools, sources of
data, other professionals, or time may be limited, making it difficult
to obtain a full and clear overview of the case. Equally, the limits of
our own experience and limits on access to supervision and consul-
tation can lead to diagnostic difficulties.

3. Cases that are controversial. For example, where there is ongoing

scientific debate about aspects of the condition, disagreement between
professionals working with the case, differences in opinion between fam-
ily members and professionals, or because the stakes are very high, such
as in several medicolegal contexts.

We have used these approximate and far from mutually exclusive categories
to structure our book. Though the term ‘diagnosis’ can be controversial,
particularly in relation to psychiatric diagnoses (e.g. Johnstone, 2018), and
though many neuropsychologists would consider their work as contributing
towards rather than solely reaching a diagnosis, we have used it in the heading
of Part 2 for a few reasons. First, we often use the term to refer to a previously
 Rare disorders in clinical neuropsychology 3
established and well-documented medical condition, such as a particular type
of stroke or other brain injury. At other times we use the term to refer to the
identification of a specifically neuropsychological syndrome, such as Balint’s
syndrome. At other times we use the term essentially as a shorthand to re-
fer to a sometimes lengthy and intricate process whereby a professional or
team of professionals collaborate with the client and often numerous other
stakeholders in order to reach a comprehensive understanding of a person’s
presentation, of which a diagnosis is one part of much broader, holistic, inter-
disciplinary formulation. Indeed, we are active proponents of psychological
and neuropsychological formulation, teaching on this topic on a number of
training courses and also having published/presented on it (e.g. Wilson &
Betteridge, 2019; Winegarder & Fish, 2017).
As a group of editors, we have considerable experience working on such
cases. Barbara Wilson in particular has a longstanding interest in rare disor-
ders. Wilson, Baddeley, and Young (1999) reported the case of LE, a 51-year-
old sculptor with systemic lupus erythematosus, an autoimmune disorder
associated with a range of cognitive impairments. LE showed only mild im-
pairments on cognitive testing, and her subjective impressions of a more se-
rious impairment that was impacting on her work were initially attributed
to anxiety and a difficulty in adjusting to the mild reductions in ability from
a previously higher level. However, LE was insistent that there was really
something the matter with her memory and noted an observation whereby
she had thought that two stained-glass windows were identical, when her
husband pointed out that they were very different. This led to more detailed
assessment of LE’s visual short-term memory and, indeed, significant prob-
lems were identified. Cases such as this were followed by a series of papers
documenting detailed assessment and intervention with people with rare
conditions including people who had emerged from disorders of conscious-
ness (e.g. Macniven et al., 2003; Wilson & Bainbridge, 2013; Wilson et al.,
2005). The Routledge book series, Survivor Stories: Life After Brain Injury,
documents further cases in detail, some entirely from the person’s own per-
spective, others in collaboration with a professional or team of professionals.
In collaboration with Michael Perdices, Barbara Wilson co-edited a spe-
cial issue of the journal Neuropsychological Rehabilitation on rare and unusual
syndromes (Perdices & Wilson, 2018), which included cases of Alice in Won-
derland syndrome, Alexander’s disease, Diogenes syndrome, Brugada syn-
drome, co-occurring Sheehan’s syndrome and sickle cell disease, and a case
of a person with a brain injury who had experienced a highly unusual ‘feral’
period during childhood.
Jessica Fish has worked in a number of highly specialist services from early
in her career, which sparked and maintained interests in rare conditions. Par-
ticularly inf luential periods included a training placement with people with
very severe brain injury at the Royal Hospital for Neuro-Disability where
a bespoke, hypothesis-driven approach to assessment and intervention was
key. She later worked at Professor Mike Kopelman’s Neuropsychiatry and
4 Introduction
Memory Disorders Service and, as Professor Kopelman is a world-leading
expert in memory disorders, people with rare conditions and unusual pres-
entations were seen frequently and benefited from the combined clinical–
academic and multidisciplinary service setting (see Kopelman & Crawford,
1996). Periods of work at the Oliver Zangwill Centre (OZC) and the Wolf-
son Neurorehabilitation Centre followed, affording the opportunity to work
intensively with clients with interacting cognitive, emotional, and physical
consequences of brain injury. During this time she joined the Professional
Panel of the excellent charity, The Encephalitis Society, whose work over the
last 20+ years has brought about huge improvements in the recognition and
treatment of this rare group of disorders. While at the OZC, she co-wrote a
paper published in the previously mentioned special issue of Neuropsychological
Rehabilitation on rare and unusual syndromes (Fish & Forrester, 2018). This
paper was notable for documenting the experience of confabulation from
the patient’s perspective, and for documenting an awareness-based interven-
tion that drew on various transferable principles from other areas of practice
but had seldom been noted in the confabulation literature. She has gone on
to supervise research on this topic in an attempt to formalise some of the
‘practice-based evidence’ that clinicians hold yet does not seem to inf luence
theory or practice to the extent that it might (Brooks et al., in prep.; Brooks,
2022), and it is hoped that this book will similarly support this endeavour.
Shai Betteridge’s experience was what directly led to the development of
the book. As head of a neuropsychology department within a busy regional
neurosciences centre, ‘rare’, ‘controversial’, and ‘difficult to diagnose’ cases
are encountered at what may seem like a paradoxically frequent rate. The
idea for the book was conceived during a supervision discussion Barbara had
with Shai. While discussing two current cases, firstly the case described in
Chapter 10 by Rose and Dilley, and secondly a case where a person’s memory
disorder appeared inconsistent with the established theories of memory (e.g.
evident learning during the day and complete lack of carryover to the follow-
ing day after a night of sleep). The case was very similar to the one described
by Smith et al. (2010) in which the patient presented with a memory profile
that had been depicted in the fictional film 50 First Dates. This case was un-
derstood to be a functional amnesia, primarily confirmed by the observed
recovery in function following psychological intervention. However, Shai’s
case (described in Chapter 24) was not responding to psychological treatment
in the way expected from the functional amnesia formulation. This led to
the reformulation of the case in order to identify alternative treatment ap-
proaches. Further exploration of the organic hypotheses that might account
for the client’s presentation has revealed fascinating hypotheses that, if con-
firmed, could revolutionise our understanding of memory profiles. Barbara
proposed that these cases ought to be published, as both challenged conven-
tional views regarding memory profiles. We considered how much we were
learning from these cases, especially in relation to the dangers of body–mind
dualism driving the misguided search for differential diagnoses in patients
 Rare disorders in clinical neuropsychology 5
with multimorbidity, and we knew that others would have similar expe-
riences. Indeed, many people consider their ‘on the job’ learning far more
inf luential in shaping their practice than the initial training (though of course
this provides the essential foundations); so the idea for this book was born.
We wanted to compile this book to inspire our professional community
to drive forward holistic models of body and mind through a comprehensive
picture of contemporary practice in clinical neuropsychology in non-routine
circumstances and less established areas of practice. We were confident that
the contents would be of interest and ref lect the discrepancies between the
empirical literature, which often concerns single diagnoses, and clinical prac-
tice, where multimorbidity and especially comorbidity of physical and men-
tal health conditions is common. Our objective was that reading this book
might speed up the rate at which people develop competence in this kind
of work. We therefore contacted our professional networks and included an
open call for chapter proposals in the newsletter of our key professional body,
the British Psychological Society (BPS) Division of Neuropsychology. The
fact that our colleagues shared our vision and responded in abundance to the
call for cases highlights how common diagnostic challenges and controversies
are in clinical practice.
Naturally the majority of the chapter authors are neuropsychologists in the
United Kingdom, but there are many chapters co-written with colleagues
who have different professional backgrounds, ref lecting the multi- and/or
interdisciplinary settings in which many of us work and which, in our view,
are crucial to providing integrated care. We are delighted that the chapters
represent people from childhood to old age. There is also considerable varia-
tion in the service contexts from which these cases are drawn. Many are from
the UK National Health Service (NHS), several are based on third sector
charitable organisations, and there are also two chapters from colleagues in
India. We think that, collectively, the chapters provide fascinating insights
into how neuropsychological principles are applied and translated – across
the lifespan, between different conditions, across the range of severity, over
myriad service settings, as well as geographical boundaries.
We hope you enjoy reading the ensuing chapters and will be back with
some concluding thoughts thereafter.

References
Bernard, S., Aspinal, F., Gridley, K., & Parker, G. (2010). Integrated services for peo-
ple with long-term neurological conditions: Evaluation of the impact of the Na-
tional Service Framework, Final Report, SPRU Working Paper No. SDO 2399,
Social Policy Research Unit, University of York, York.
British Psychological Society (2019a). Standards for the accreditation of Doctoral
programmes in clinical psychology. Leicester, BPS.
British Psychological Society (2019b). Standards for the accreditation of programmes
in adult clinical neuropsychology. Leicester, BPS.
6 Introduction
Brooks, E. (2022). Exploring the clinical management of confabulation within neu-
ropsychology services. D Clin Psy thesis, University of Glasgow.
Brooks, E., Evans, J., & Fish, J. (in preparation). The clinical management of confabulation
in neuropsychology services: A practitioner survey and interview study.
Fish, J., & Forrester, J. (2018). Developing awareness of confabulation through psy-
chological formulation: A case report and first-person perspective. Neuropsycholog-
ical Rehabilitation, 28(2), 277–292.
Johnstone, L. (2018). Psychological formulation as an alternative to psychiatric diag-
nosis. Journal of Humanistic Psychology, 58(1), 30–46.
Macniven, J. A., Poz, R., Bainbridge, K., Gracey, F., & Wilson, B. A. (2003). Emo-
tional adjustment following cognitive recovery from ‘persistent vegetative state’:
Psychological and personal perspectives. Brain Injury, 17(6), 525–533.
Kopelman, M., & Crawford, S. (1996). Not all memory clinics are dementia clinics.
Neuropsychological Rehabilitation, 6(3), 187–202.
Macniven, J. A., Poz, R., Bainbridge, K., Gracey, F., & Wilson, B. A. (2003). Emo-
tional adjustment following cognitive recovery from ‘persistent vegetative state’:
Psychological and personal perspectives. Brain Injury, 17(6), 525–533.
Marshall, J. C., & Gurd, J. M. (2010). Neuropsychology: Past, present, and fu-
ture. In J. Gurd & U. Kischka (Eds), The handbook of clinical neuropsychology,
2nd edn. Oxford: Oxford Academic, online edition. https://doi.org/10.1093/
acprof:oso/9780199234110.003.01
Perdices, M., & Wilson, B. A. (2018). Introduction to the special issue on rare and
unusual syndromes. Neuropsychological Rehabilitation, 28(2), 185–188.
Shallice, T. (1988). From neuropsychology to mental structure. Cambridge University
Press.
Smith, C. N., Frascino, J. C., Kripke, D. L., McHugh, P. R., Treisman, G. J., &
Squire, L. R. (2010). Losing memories overnight: A unique form of human amne-
sia. Neuropsychologia, 48(10), 2833–2840.
Vallar, G., & Caputi, N. (2020). The history of human neuropsychology. In S. Della
Sala (Ed.), Encyclopedia of behavioral neuroscience (2nd ed.). Elsevier. https://doi.
org/10.1016/B978-0-12-809324-5.23914-X
Wilson, B. A., Baddeley, A. D., & Young, A. W. (1999). L.E.: A person who lost her
‘Mind’s Eye’. Neurocase, 5, 119–127.
Wilson, B. A., & Bainbridge, K. (2013). Kate’s story: Recovery takes time, so don’t
give up. In B. A. Wilson, J. Winegardner, & F. Ashworth (Eds.), Life after brain
injury (pp. 68–80). Psychology Press.
Wilson, B. A., Berry, E., Gracey, F., Harrison, C., Stow, I., Macniven, J., … &
Young, A. W. (2005). Egocentric disorientation following bilateral parietal lobe
damage. Cortex, 41(4), 547–554.
Wilson, B. A., & Betteridge, S. (2019). Essentials of neuropsychological rehabilitation.
Guilford Publications.
Wilson, B. A., Gracey, F., Evans, J. J., & Bateman, A. (2009). Neuropsychological reha-
bilitation: Theory, models, therapy and outcome. Cambridge University Press.
Winegardner, J., & Fish, J. E. (2017, July). A novel approach to interdisciplinary team as-
sessment: Joining the dots. Abstract of datablitz presentation at the World Federation
of NeuroRehabilitation (WFNR) 14th annual conference on neuropsychological
rehabilitation, Cape Town, South Africa.
Part 1

Rare and unusual

conditions
2 Gas geyser syndrome in India
A tragic, preventable
neuropsychological morbidity
Urvashi Shah

Introduction
It often happens that the real tragedies of life occur in such an inartistic manner that
they hurt us by their crude violence, their absolute incoherence, their absurd want
of meaning, their entire lack of style.
(Oscar Wilde, The Picture of Dorian Gray,
ed. J. Bistrow (2005), p. 78)

‘Gas geyser syndrome’ (GGS) is a known phenomenon to the medical fra-

ternity in India. Although infrequent, cases are seen in emergency units and
many physicians have become familiar with the history and pattern of symp-
tom presentation. However, the diagnosis can still be confounding as many
symptoms may be similar to those seen after exposure to other gases. Various
published case reports have underscored the need for a careful history and re-
view of imaging findings to reach a conclusive diagnosis (Anand et al., 2006;
Correia et al., 2012; Mehta et al., 2016). Gas geyser syndrome has also been
reported in other South Asian countries such as Pakistan (Quasim, 2017),
Nepal (Bista et al., 2017), and BRICS countries (Brazil, Russia, India, China,
and South Africa) (Sampson, 2017) where erratic electricity supply and the
low cost of gas geysers makes them a popular choice.
In western countries, common causes of carbon monoxide (CO) toxicity
are exhaust from engines, fire (smoke inhalation), and poorly installed fur-
naces. Flue-less (no duct/chimney) gas heaters are cheaper to run as compared
to electric fires and are easy to install. In India, the commonest cause of CO
poisoning is the use of faulty gas appliances for cooking in enclosed spaces,
especially in the winter months (Sikary et al., 2017).
Gas geysers are connected to liquefied petroleum gas (LPG) cylinders.
LPG contains C3 and C4 hydrocarbons and the gas contains butane and pro-
pane. The gas is heated by a gas burner and is delivered by an inlet pipe.
When there is proper combustion, carbon dioxide and water are produced.
As the gas heats and pressure is built up, the safety pressure valve in the unit
discharges the pressure. An attractive feature of this geyser is that there is an
instant and continuous supply of heated water and this appeals to people liv-
ing in areas where there are frequent power cuts and harsh winters.

DOI: 10.4324/9781003228226-3
10 Rare and unusual conditions
However, in many homes in India, there are small bathing spaces with
poor cross-ventilation and in winter families tend to keep the windows shut,
resulting in poorly ventilated bathrooms with insufficient oxygen. In these
spaces, an incomplete combustion results in the formation of the toxic, lethal
carbon monoxide gas.
Despite several media reports of death due to carbon monoxide poison-
ing after using a gas geyser, and case reports in the Indian medical litera-
ture, the gas geyser continues to be popular and there appears to be little or
no awareness about the precautions that need to be taken while using this
geyser.
There have been no guidelines about the safe use of gas geysers and it is
only recently that in India a government gas agency has brought out a doc-
ument listing recommendations for the proper installation of these geysers
(Mahanagar Gas Limited, 2020).
CO is an odourless, colourless, tasteless, non-irritant gas that binds to the
hemoglobin creating carboxyhemoglobin (COHb). Hemoglobin (Hb) has a
very high affinity to bind with CO, almost 250 times higher than oxygen.
COHb reduces the capacity of the blood to bind oxygen, thereby decreasing
the oxygen transport mechanism and delivery of oxygen to the tissues, thus
causing hypoxia. Additionally, the exposure to CO brings about mitochon-
drial inhibition and free radical generation resulting in ischemic and anoxic
brain injury that causes the cognitive deficits (Rose et al., 2017). Certain
organs such as the heart and the brain that have a greater requirement of
oxygen are more vulnerable than other organ systems. The most common
imaging findings are white matter hyperintensities and hippocampal damage
(Weaver et al., 2015; Parkinson et al., 2002). The magnitude and spectrum
of symptoms vary according to the degree of exposure-concentration of CO
and duration of exposure. Several patients have been found in an unconscious
state. Increasing concentrations of COHb, ranging from 10% to 60%, are
associated with different presenting symptoms such as headaches and dizzi-
ness, altered mentation and cognitive deficits, and at higher percentages, to
seizures, coma, or even death.
It has been suggested that even low level exposure, but of longer dura-
tion, can result in significant cognitive issues (Townsend & Maynard, 2002).
Long-term problems (> 6 years) have been reported in a subgroup of patients
suggesting irreversibility in some people (Weaver et al., 2008). The quality
of life in these survivors is significantly impacted in the long term with per-
sisting cognitive and mood issues (Pages et al., 2014). Depression and anxiety
are common and occur independently of the severity of the poisoning (Chel-
sea et al., 2007).
In terms of management, the current recommendation, although not man-
dated, is either hyperbaric (HbO2) or normobaric (NBO2) oxygen as soon as
possible (Wolf et al., 2008). A double blind, randomized trial for HbO2 has
shown that hyperbaric-oxygen within the first 24 hours has benefit on long-
term cognitive outcomes (Weaver et al, 2002).
Another random document with
no related content on Scribd:
more recent invention, called the Belgrave
mould (which is to be had of the originators,
Messrs. Temple and Reynolds, Princes
Street, Cavendish Square, and also at 80,
Motcomb Street, Belgrave Square), is of
superior construction for the purpose, as it
contains a large central cylinder and six
smaller ones, which when withdrawn, after
the jelly—which should be poured round,
but not into them—is set, leave vacancies
which can be filled either with jelly of another colour, or with fruit of
different kinds (which must be secured in its place with just liquid
jelly poured carefully in after it is arranged), or with blanc-mange, or
any other isinglass-cream. The space occupied by the larger cylinder
may be left empty, or filled, before the jelly is served, with white or
with pale-tinted whipped cream. Water, only sufficiently warm to
detach the jelly from them without heating or melting it, must be
poured into the cylinders to unfix them; and to loosen the whole so
as to unmould it easily, a cloth wrung out of very hot water must be
wound round it, or the mould must be dipped quickly into some
which is nearly or quite boiling. A dish should then be laid on it, it
should be carefully reversed, and the mould lifted from it gently. It will
sometimes require a slight sharp blow to detach it quite.
Italian jelly is made by half filling a mould of convenient form, and
laying round upon it in a chain, as soon as it is set, some blanc-
mange made rather firm, and cut of equal thickness and size, with a
small round cutter; the mould is then filled with the remainder of the
jelly, which must be nearly cold, but not beginning to set. Branched
morella cherries, drained very dry, are sometimes dropped into
moulds of pale jelly; and fruits, either fresh or preserved, are
arranged in them with exceedingly good effect when skilfully
managed; but this is best accomplished by having a mould for the
purpose, with another of smaller size fixed in it by means of slight
wires, which hook on to the edge of the outer one. By pouring water
into this it may easily be detached from the jelly; the fruit is then to
be placed in the space left by it, and the whole filled up with more
jelly: to give the proper effect, it must be recollected that the dish will
be reversed when sent to table.
 QUEEN MAB’S PUDDING.

(An Elegant Summer Dish.)

Throw into a pint of new milk the thin rind of a small lemon, and six
or eight bitter almonds, blanched and bruised; or substitute for these
half a pod of vanilla cut small, heat it slowly by the side of the fire,
and keep it at the point of boiling until it is strongly flavoured, then
add a small pinch of salt, and three-quarters of an ounce of the finest
isinglass, or a full ounce should the weather be extremely warm;
when this is dissolved, strain the milk through a muslin, and put it
into a clean saucepan, with from four to five ounces and a half of
sugar in lumps, and half a pint of rich cream; give the whole one boil,
and then stir it, briskly and by degrees, to the well-beaten yolks of six
fresh eggs; next, thicken the mixture as a custard, over a gentle fire,
but do not hazard its curdling; when it is of tolerable consistence,
pour it out, and continue the stirring until it is half cold, then mix with
it an ounce and a half of candied citron, cut in small spikes, and a
couple of ounces of dried cherries, and pour it into a mould rubbed
with a drop of oil: when turned out it will have the appearance of a
pudding. From two to three ounces of preserved ginger, well drained
and sliced, may be substituted for the cherries, and an ounce of
pistachio-nuts, blanched and split, for the citron; these will make an
elegant variety of the dish, and the syrup of the ginger, poured round
as sauce, will be a further improvement. Currants steamed until
tender, and candied orange or lemon-rind, are often used instead of
the cherries, and the well-sweetened juice of strawberries,
raspberries (white or red), apricots, peaches, or syrup of pine-apple,
will make an agreeable sauce; a small quantity of this last will also
give a delicious flavour to the pudding itself, when mixed with the
other ingredients. Cream may be substituted entirely for the milk,
when its richness is considered desirable.
New milk, 1 pint; rind 1 small lemon; bitter almonds, 6 to 8 (or,
vanilla, 1/2 pod); salt, few grains; isinglass, 3/4 oz. (1 oz. in sultry
weather); sugar, 4-1/2 oz.; cream, 1/2 pint; yolks, 6 eggs; dried
cherries, 2 oz.; candied citron, 1-1/2 oz.; (or, preserved ginger, 2 to 3
oz., and the syrup as sauce, and 1 oz. of blanched pistachio-nuts; or
4 oz. currants, steamed 20 minutes, and 2 oz. candied orange-rind).
For sauce, sweetened juice of strawberries, raspberries, or plums, or
pine apple syrup.
Obs.—The currants should be steamed in an earthen cullender,
placed over a saucepan of boiling water, and covered with the lid. It
will be a great improvement to place the pudding over ice for an hour
before it is served.
 NESSELRÔDE CREAM.

Shell and blanch (see page 342) twenty-four fine Spanish

chestnuts, and put them with three-quarters of a pint of water into a
small and delicately clean saucepan. When they have simmered
from six to eight minutes, add to them two ounces of fine sugar, and
let them stew very gently until they are perfectly tender; then drain
them from the water, pound them, while still warm, to a smooth
paste, and press them through the back of a fine sieve. While this is
being done, dissolve half an ounce of isinglass in two or three
spoonsful of water, and put to it as much cream as will, with the
small quantity of water used, make half a pint, two ounces of sugar,
about the third of a pod of vanilla, cut small, and well bruised, and a
strip or two of fresh lemon-rind, pared extremely thin. Give these a
minute’s boil, and then keep them quite hot by the side of the fire,
until a strong flavour of the vanilla is obtained. Now, mix gradually
with the chestnuts half a pint of rich, unboiled cream, strain the other
half pint through a fine muslin, and work the whole well together until
it becomes very thick; then stir to it a couple of ounces of dried
cherries, cut into quarters, and two of candied citron, divided into
very small dice. Press the mixture into a mould which has been
rubbed with a particle of the purest salad-oil, and in a few hours it will
be ready for table. The cream should be sufficiently stiff, when the
fruit is added, to prevent its sinking to the bottom, and both kinds
should be dry when they are used.
Chestnuts, large, 24; water, 3/4 pint; sugar, 2 oz.; isinglass, 1/2
oz.; water, 3 to 4 tablespoonsful; cream, nearly 1/2 pint; vanilla, 1/3
of pod; lemon-rind, 1/4 of 1 large: infuse 20 minutes or more.
Unboiled cream, 1/2 pint; dried cherries, 2 oz.; candied citron, 2 oz.
Obs.—When vanilla cannot easily be obtained, a little noyau may
be substituted for it, but a full weight of isinglass must then be used.
 CRÊME À LA COMTESSE, OR THE COUNTESS’S CREAM.

Prepare as above, boil and pound, eighteen fine sound chestnuts;

mix with them gradually, after they have been pressed through a fine
sieve, half a pint of rich sweet cream; dissolve in half a pint of new
milk a half-ounce of isinglass, then add to them from six to eight
bitter almonds, blanched and bruised, with two-thirds of the rind of a
small lemon, cut extremely thin, and two ounces and a half of sugar;
let these simmer gently for five minutes, and then remain by the side
of the fire for awhile. When the milk is strongly flavoured, strain it
through muslin, press the whole of it through, and stir it by degrees
to the chestnuts and cream; beat the mixture smooth, and when it
begins to thicken, put it into a mould rubbed with oil, or into one
which has been dipped in water and shaken nearly free of the
moisture. If set into a cool place, it will be ready for table in six or
eight hours. It has a pretty appearance when partially stuck with
pistachio-nuts, blanched, dried, and cut in spikes, their bright green
colour rendering them very ornamental to dishes of this kind: as they
are, however, much more expensive than almonds, they can be used
more sparingly, or intermingled with spikes of the firm outer rind of
candied citron.
Chestnuts, 18; water, full 1/2 pint; sugar, 1 oz.: 15 to 25 minutes,
or more. Cream, 1/2 pint; new milk, 1/2 pint; isinglass, 1/2 oz.; bitter
almonds, 6 to 8; lemon-rind, two-thirds of 1; sugar, 2-1/2 oz.[161]
161. The proportions both of this and of the preceding cream must be increased
for a large mould.

Obs.—This is a very delicate kind of sweet dish, which we can

particularly recommend to our readers; it may be rendered more
recherché by a flavouring of maraschino, but must then have a little
addition of isinglass. The preparation, without this last ingredient, will
be found excellent iced.
 AN EXCELLENT TRIFLE.

Take equal parts of wine and brandy, about a wineglassful of each,

or two-thirds of good sherry or Madeira, and one of spirit, and soak
in the mixture four sponge-biscuits, and half a pound of macaroons
and ratifias; cover the bottom of the trifle-dish with part of these, and
pour upon them a full pint of rich boiled custard made with three-
quarters of a pint, or rather more, of milk and cream taken in equal
portions, and six eggs; and sweetened, flavoured and thickened by
the receipt of page 481; lay the remainder of the soaked cakes upon
it, and pile over the whole, to the depth of two or three inches, the
whipped syllabub of page 476, previously well drained; then sweeten
and flavour slightly with wine only, less than half a pint of thin cream
(or of cream and milk mixed); wash and wipe the whisk, and whip it
to the lightest possible froth: take it off with a skimmer and heap it
gently over the trifle.
Macaroons and ratifias, 1/2 lb.; wine and brandy mixed, 1/4 pint;
rich boiled custard, 1 pint; whipped syllabub (see page 476); light
froth to cover the whole, short 1/2 pint of cream and milk mixed;
sugar, dessertspoonful; wine, 1/2 glassful.
 SWISS CREAM, OR TRIFLE.

(Very Good.)
Flavour pleasantly with and cinnamon, a pint of rich cream, after
having taken from it as much as will mix smoothly to a thin batter
four teaspoonsful of the finest flour; sweeten it with six ounces of
well-refined sugar in lumps; place it over a clear fire in a delicately
clean saucepan, and when it boils stir in the flour, and simmer it for
four or five minutes, stirring it gently without ceasing; then pour it out,
and when it is quite cold mix with it by degrees the strained juice of
two moderate-sized and very fresh lemons. Take a quarter of a
pound of macaroons, cover the bottom of a glass dish with a portion
of them, pour in a part of the cream, lay the remainder of the
macaroons upon it, add the rest of the cream, and ornament it with
candied citron sliced thin. It should be made the day before it is
wanted for table. The requisite flavour may be given to this dish by
infusing in the cream the very thin rind of a lemon, and part of a stick
of cinnamon slightly bruised, and then straining it before the flour is
added; or, these and the sugar may be boiled together with two or
three spoonsful of water, to a strongly flavoured syrup, which, after
having been passed through a muslin strainer, may be stirred into
the cream. Some cooks boil the cinnamon and the grated rind of a
lemon with all the other ingredients, but the cream has then to be
pressed through a sieve after it is made, a process which it is always
desirable to avoid. It may be flavoured with vanilla and maraschino,
or with orange-blossoms at pleasure; but is excellent made as
above.
Rich cream, 1 pint; sugar, 6 oz.; rind, 1 lemon; cinnamon, 1
drachm; flour, 4 teaspoonsful; juice, 2 lemons; macaroons, 4 oz.;
candied citron, 1 to 2 oz.
 TIPSY CAKE, OR BRANDY TRIFLE.

The old-fashioned mode of preparing this dish was to soak a light

sponge or Savoy cake in as much good French brandy as it could
absorb; then, to stick it full of blanched almonds cut into whole-length
spikes, and to pour a rich cold boiled custard round it. It is more
usual now to pour white wine over the cake, or a mixture of wine and
brandy; with this the juice of half a lemon is sometimes mixed.

Chantilly Basket.
 FILLED WITH WHIPPED CREAM AND FRESH STRAWBERRIES.

Take a mould of any sort that will serve to form the basket on, just
dip the edge of some macaroons into melted barley sugar, and
fasten them together with it; take it out of the mould, keep it in a dry
place until wanted, then fill it high with whipped strawberry cream
which has been drained on a sieve from the preceding day, and stick
very fine ripe strawberries over it. It should not filled until just before
it is served.
 VERY GOOD LEMON CREAMS MADE WITHOUT CREAM.

Pour over the very thin rinds of two moderate-sized but perfectly
sound fresh lemons and six ounces of sugar, half a pint of spring
water, and let them remain for six hours: then add the strained juice
of the lemons, and five fresh eggs well beaten and also strained;
take out the lemon-rind, and stir the mixture without ceasing over a
gentle fire until it has boiled softly from six to eight minutes: it will not
curdle as it would did milk supply the place of the water and lemon-
juice. The creams are, we think, more delicate, though not quite so
thick, when the yolks only of six eggs are used for them. They will
keep well for nearly a week in really cold weather.
Rinds of lemons, 2; sugar, 6 oz. (or 8 when a very sweet dish is
preferred); cold water, 1/2 pint: 6 hours. Juice of lemons, 2; eggs, 5:
to be boiled softly 6 to 8 minutes.
Obs.—Lemon creams may, on occasion, be more expeditiously
prepared, by rasping the rind of the fruit upon the sugar which is
used for them; or, by paring it thin, and boiling it for a few minutes
with the lemon-juice, sugar, and water, before they are stirred to the
eggs.
 FRUIT CREAMS, AND ITALIAN CREAMS.

These are very quickly and easily made, by mixing with good
cream a sufficient proportion of the sweetened juice of fresh fruit, or
of well-made fruit jelly or jam, to flavour it: a few drops of prepared
cochineal may be added to deepen the colour when it is required for
any particular purpose. A quarter of a pint of strawberry or of
raspberry jelly will fully flavour a pint of cream: a very little lemon-
juice improves almost all compositions of this kind. When jam is
used it must first be gradually mixed with the cream, and then
worked through a sieve, to take out the seed or skin of the fruit. All
fresh juice, for this purpose, must of course, be cold; that of
strawberries is best obtained by crushing the fruit and strewing sugar
over it. Peaches, pine-apple, apricots, or nectarines, may be
simmered for a few minutes in a little syrup, and this, drained well
from them, will serve extremely well to mix with the cream when it
has become thoroughly cold: the lemon-juice should be added to all
of these. When the ingredients are well blended, lightly whisk or mill
them to a froth; take this off with a skimmer as it rises, and lay it
upon a fine sieve reversed, to drain, or if it is to be served in glasses,
fill them with it at once.
Italian creams are either fruit-flavoured only, or mixed with wine
like syllabubs, then whisked to a stiff froth and put into a perforated
mould, into which a muslin is first laid; or into a small hair-sieve
(which must also first be lined with the muslin), and left to drain until
the following day, when the cream must be very gently turned out,
and dished, and garnished, as fancy may direct.
 VERY SUPERIOR WHIPPED SYLLABUBS.

Weigh seven ounces of fine sugar and rasp on it the rinds of two
fresh sound lemons of good size, then pound or roll it to powder, and
put it into a bowl with the strained juice of the lemons, two large
glasses of sherry, and two of brandy; when the sugar is dissolved
add a pint of very fresh cream, and whisk or mill the mixture well;
take off the froth as it rises, and put it into glasses. These syllabubs
will remain good for several days, and should always be made if
possible, four-and-twenty hours before they are wanted for table.
The full flavour of the lemon-rind is obtained with less trouble than in
rasping, by paring it very thin indeed, and infusing it for some hours
in the juice of the fruit.
Sugar, 7 oz.; rind and juice of lemons, 2; sherry, 2 large
wineglassesful; brandy, 2 wineglassesful; cream, 1 pint.
Obs.—These proportions are sufficient for two dozens or more of
syllabubs: they are often made with almost equal quantities of wine
and cream, but are considered less wholesome without a portion of
brandy.
 BLANC-MANGES.

GOOD COMMON BLANC-MANGE, OR BLANC-MANGER.

(Author’s Receipt.)
Infuse for an hour in a pint
and a half of new milk the very
thin rind of one small, or of half a
large lemon and four or five
bitter almonds, blanched and
bruised,[162] then add two
ounces of sugar, or rather more
for persons who like the blanc-
mange very sweet, and an
ounce and a half of isinglass. Blanc-mange or Cake Mould.
Boil them gently over a clear
fire, stirring them often until this last is dissolved; take off the scum,
stir in half a pint, or rather more, of rich cream, and strain the blanc-
mange into a bowl; it should be moved gently with a spoon until
nearly cold to prevent the cream from settling on the surface. Before
it is moulded, mix with it by degrees a wineglassful of brandy.
162. These should always be very sparingly used.
New milk, 1-1/2 pint; rind of lemon, 1/2 large or whole small; bitter
almonds, 8: infuse 1 hour. Sugar, 2 to 3 oz.; isinglass, 1-1/2 oz.: 10
minutes. Cream, 1/2 pint; brandy, 1 wineglassful.
 RICHER BLANC-MANGE.

A pint of good cream with a pint of new milk, sweetened and

flavoured as above (or in any other manner which good taste may
dictate), with a little additional sugar, and the same proportion of
isinglass, will make very good blanc-mange. Two ounces of Jordan
almonds may be pounded and mixed with it, but they are not needed
with the cream.
JAUMANGE, OR JAUNE MANGER, SOMETIMES CALLED DUTCH
FLUMMERY.

Pour on the very thin rind of a large lemon and half a pound of
sugar broken small, a pint of water, and keep them stirred over a
gentle fire until they have simmered for three or four minutes, then
leave the saucepan by the side of the stove that the syrup may taste
well of the lemon. In ten or fifteen minutes afterwards add two
ounces of isinglass, and stir the mixture often until this is dissolved,
then throw in the strained juice of four sound moderate-sized
lemons, and a pint of sherry; mix the whole briskly with the beaten
yolks of eight fresh eggs, and pass it through a delicately clean hair-
sieve: next thicken it in a jar or jug placed in a pan of boiling water,
turn it into a bowl, and when it has become cool and been allowed to
settle for a minute or two, pour it into moulds which have been laid in
water. Some persons add a small glass of brandy to it, and deduct
so much from the quantity of water.
Rind of 1 lemon; sugar, 8 oz.; water, 1 pint: 3 or 4 minutes.
Isinglass, 2 oz.; juice, 4 lemons; yolks of eggs, 8; wine, 1 pint;
brandy (at pleasure), 1 wineglassful.
 EXTREMELY GOOD STRAWBERRY BLANC-MANGE, OR
BAVARIAN CREAM.

Crush slightly with a silver or wooden spoon, a quart, measured

without their stalks, of fresh and richly-flavoured strawberries; strew
over them eight ounces of pounded sugar, and let them stand for
three or four hours; then turn them on to a fine hair-sieve reversed,
and rub them through it. Melt over a gentle fire two ounces of the
best isinglass in a pint of new milk, and sweeten it with four ounces
of sugar; strain it through a muslin, and mix it with a pint and a
quarter of sweet thick cream; keep these stirred until they are nearly
or quite cold, then pour them gradually to the strawberries, whisking
them briskly together; and last of all throw in, by small portions, the
strained juice of a fine sound lemon. Mould the blanc-mange, and
set it in a very cool place for twelve hours or more before it is served.
Strawberries stalked, 1 quart; sugar, 8 oz.; isinglass, 2 oz.; new
milk, 1 pint; sugar, 4 oz.; cream, 1-1/4 pint; juice, 1 lemon.
Obs.—We have retained here the old-fashioned name of blanc-
mange (or blanc-manger) because it is more familiar to many
English readers than any of recent introduction; but moulded
strawberry-cream would be more appropriate; as nothing can
properly be called blanc manger which is not white. By mingling the
cream, after it has been whisked, or whipped, to the other
ingredients, the preparation becomes what is called un Fromage
Bavarois, or Bavarian cream, sometimes simply, une Bavaroise.
 QUINCE BLANC-MANGE.

(Delicious.)
This, if carefully made, and with ripe quinces, is one of the most
richly-flavoured preparations of fruit that we have ever tasted; and
the receipt, we may venture to say, will be altogether new to the
reader. Dissolve in a pint of prepared juice of quinces (see page
456), an ounce of the best isinglass; next, add ten ounces of sugar,
roughly pounded, and stir these together gently over a clear fire,
from twenty to thirty minutes, or until the juice jellies in falling from
the spoon. Remove the scum carefully, and pour the boiling jelly
gradually to half a pint of thick cream, stirring them briskly together
as they are mixed: they must be stirred until very nearly cold, and
then poured into a mould which has been rubbed in every part with
the smallest possible quantity of very pure salad oil, or if more
convenient, into one that has been dipped into cold water.
Obs.—This blanc-manger which we had made originally on the
thought of the moment for a friend, proved so very rich in flavour,
that we inserted the exact receipt for it, as we had had it made on
our first trial; but it might be simplified by merely boiling the juice,
sugar, and isinglass, together for a few minutes, and then mixing
them with the cream. An ounce and a half of isinglass and three-
quarters of a pint of cream might then be used for it. The juice of
other fruit may be substituted for that of the quinces.
Juice of quinces, 1 pint; isinglass, 1 oz.: 5 to 10 minutes. Sugar, 10
oz.: 20 to 30 minutes. Cream, 1/2 pint.
 QUINCE BLANC-MANGE, WITH ALMOND CREAM.

When cream is not procurable, which will sometimes happen in

the depth of winter, almonds, if plentifully used, will afford a very
good substitute, though the finer blanc-mange is made from the
foregoing receipt. On four ounces of almonds, blanched and beaten
to the smoothest paste, and moistened in the pounding with a few
drops of water, to prevent their oiling, pour a pint of boiling quince-
juice; stir them together, and turn them into a strong cloth, of which
let the ends be held and twisted different ways by two persons, to
express the cream from the almonds; put the juice again on the fire,
with half a pound of sugar, and when it boils, throw in nearly an
ounce of fine isinglass; simmer the whole for five minutes, take off
the scum, stir the blanc-mange until it is nearly cold, then mould it for
table. Increase the quantity both of this and of the preceding blanc-
mange, when a large dish of either is required.
Quince-juice, 1 pint; Jordan almond, 4 oz.; sugar, 1/2 lb; isinglass,
nearly 1 oz: 5 minutes.
 APRICOT BLANC-MANGE, OR CRÊME PARISIENNE.

Dissolve gently an ounce of fine isinglass in a pint of new milk or

of thin cream, and strain it through a folded muslin; put it into a clean
saucepan, with three ounces of sugar, broken into small lumps, and
when it boils, stir to it half a pint of rich cream; add it, at first by
spoonsful only, to eight ounces of the finest apricot jam, mix them
very smoothly, and stir the whole until it is nearly cold that the jam
may not sink to the bottom of the mould: a tablespoonful of lemon-
juice will improve the flavour.
When cream is scarce, use milk instead, with an additional quarter
of an ounce of isinglass, and enrich it by pouring it boiling on the
same proportion of almonds as for the second quince blanc-mange
(see page 478). Cream can in all cases be substituted entirely for the
milk, when a very rich preparation is desired. Peach jam will answer
admirably for this receipt; but none of any kind should be used for it
which has not been passed through a sieve when made.
Isinglass, 1 oz.; new milk, 1 pint; cream, 1/2 pint; sugar, 3 oz.;
apricot jam, 1/2 lb.; lemon-juice, 1 tablespoonful. Or, peach jam, 1/2
lb.; cream, 1-1/2 pint.