Autoimmune Encephalitis and Related

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AUTOIMMUNE
ENCEPHALITIS
and Related Disorders of
the Nervous System

OMHftlJH- K M«dkin»
Autoimmune Encephalitis and Related Disorders of the Nervous System
Published online by Cambridge University Press: 27 January 2022

Josep Dalmau and

Francesc Graus

Autoimmune Encephalitis and Related Disorders of the Nervous System

Josep Dalmau

Catalan Institution for Research and Advanced Studies (ICREA), Department of

Neurology, Hospital Clínic, and Institut d’Investigacions Biomèdiques August Pi i Sunyer
(IDIBAPS), University of Barcelona

Francesc Graus

Department of Neurology, Hospital Clínic, and Institut d’Investigacions Biomèdiques

August Pi i Sunyer (IDIBAPS), Barcelona
Copyright page
Published online by Cambridge University Press: 27 January 2022

Josep Dalmau and

Francesc Graus

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DOI: 10.1017/9781108696722

© Cambridge University Press 2022

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without the written permission of Cambridge University Press.

First published 2022

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A catalogue record for this publication is available from the British Library.

Library of Congress Cataloging-in-Publication Data

Names: Dalmau, Josep, 1953– author. | Graus, Francesc, 1953- author.

Title: Autoimmune encephalitis and related disorders of the nervous

system / Josep Dalmau, Francesc Graus.

Description: Cambridge, United Kingdom ; New York, NY : Cambridge

University Press, 2022. | Includes bibliographical references and index.

Identifiers: LCCN 2021027938 (print) | LCCN 2021027939 (ebook) | ISBN

9781316514245 (hardback) | ISBN 9781108696722 (ebook)

Subjects: MESH: Encephalitis – immunology | Encephalomyelitis – immunology |

Autoimmune Diseases of the Nervous System

Classification: LCC RC141.E6 (print) | LCC RC141.E6 (ebook) | NLM WL 351

| DDC 616.8/32079–dc23

LC record available at https://lccn.loc.gov/2021027938

LC ebook record available at https://lccn.loc.gov/2021027939

ISBN 978-1-316-51424-5 Hardback

ISBN 9781108696722 Cambridge Core

ISBN 9781108583510 Print/Online Bundle

Additional resources for this publication at www.cambridge.org/9781108583510

Cambridge University Press has no responsibility for the persistence or accuracy of URLs
for external or third-party internet websites referred to in this publication and does not
guarantee that any content on such websites is, or will remain, accurate or appropriate.

Every effort has been made in preparing this book to provide accurate and up-to-date
information that is in accord with accepted standards and practice at the time of
publication. Although case histories are drawn from actual cases, every effort has been
made to disguise the identities of the individuals involved. Nevertheless, the authors,
editors, and publishers can make no warranties that the information contained herein is
totally free from error, not least because clinical standards are constantly changing
through research and regulation. The authors, editors, and publishers therefore disclaim
all liability for direct or consequential damages resulting from the use of material
contained in this book. Readers are strongly advised to pay careful attention to
information provided by the manufacturer of any drugs or equipment that they plan to
use.
Dedication
Published online by Cambridge University Press: 27 January 2022

Josep Dalmau and

Francesc Graus

To Myrna, Flora, Alba, and Andrea

For being at our sides in good and bad times

Contents
Published online by Cambridge University Press: 27 January 2022

Josep Dalmau and

Francesc Graus

Contents

1. List of Clinical Vignettes

2. List of Videos

3. Preface

4. List of Abbreviations

5. Section 1Overview

1. 1Importance, Definitions, History, Classification, and Frequency of the Autoimmune

Encephalitides

2. 2General Approach to Diagnosis

3. 3Pathogenesis and Disease Mechanisms in Neuronal Antibody-Mediated

Encephalitis

6. Section 2Antibodies and Antigens

1. 4Antibodies to Intracellular Antigens in CNS Disorders

2. 5Antibodies to Neural Cell Surface Antigens

7. Section 3Specific Syndromes and Diseases

1. 6Limbic Encephalitis
 2. 7Autoimmunity Against Proteins Associated with Voltage-Gated Potassium
Channels

3. 8Anti-NMDAR Encephalitis

4. 9Seizures and Antibodies Against Surface Antigens

5. 10Acute Disseminated Encephalomyelitis and Myelin Oligodendrocyte Glycoprotein

Antibody-Associated Disease

6. 11Neuromyelitis Optica Spectrum Disorders and Glial Fibrillary Acidic Protein

Autoimmunity

7. 12Autoimmune Cerebellar Ataxias

8. 13Autoimmune Brainstem Encephalitis

9. 14Autoimmunity Against the Inhibitory Synapsis

10. 15Anti-IgLON5 Disease

11. 16Autoimmune and Inflammatory Encephalopathies as Complications of Cancer

12. 17Deconstructing Hashimoto Encephalopathy

13. 18CNS Syndromes at the Frontier of Autoimmune Encephalitis

8. Section 4Autoimmunity in Neurological and Psychiatric Diseases

1. 19Autoimmune Psychosis

2. 20Psychiatric Manifestations of Autoimmune Encephalitis

3. 21Abnormal Movements in Neurological Autoimmune Disorders

4. 22Sleep and Autoimmunity

 5. 23Immunity, Inflammation, and Epilepsy

6. 24Autoimmune Dementia: A Useful Term?

7. 25Frequently Asked Questions on Autoimmune Encephalitis and Related Disorders

9. Index
Clinical Vignettes
Published online by Cambridge University Press: 27 January 2022

Josep Dalmau and

Francesc Graus

(In alphabetical order of the antibody or neurological syndrome)

Topic Number Page
AK5 limbic encephalitis 2.3 36
AMPAR encephalitis, and psychosis 20.3 539
AMPAR encephalitis and breast cancer 5.1 140
Amphiphysin antibodies, spinal 21.3 554
myoclonus, and small-cell lung cancer
AQP4 antibodies and encephalitis 11.2 322
AQP4 antibodies, area postrema 11.1 321
syndrome, and lung cancer
Behget disease and brainstem 18.1 479
encephalitis
Bickerstaff brainstem encephalitis 13.2 374
CART cell neurotoxicity 16.3 448
CASPR2 antibodies and cerebellar 7.4 201
ataxia
CASPR 2 antibodies and dementia 24.1 619
CASPR2 antibodies and Morvan 7.5 204
syndrome
CASPR 2 encephalitis presenting with 7.3 201
orthostatic tremor
cerebral amyloid angiopathy-related 18.4 492
inflammation
CV2 (CRMP5) antibodies, cerebellar 12.2 350
ataxia, and lung cancer
CV2 (CRMP5) antibodies, optic 10.4 307
neuropathy, and small-cell lung cancer
Dipeptidyl-peptidase-like protein 6 14.3 404
(DPPX) encephalitis, an autopsy study
DPPX encephalitis, index case 5.2 151
Topic Number Page

AK5 limbic encephalitis 2.3 36

AMPAR encephalitis, and

20.3 539
psychosis

AMPAR encephalitis and

5.1 140
breast cancer

Amphiphysin antibodies,
spinal myoclonus, and 21.3 554
small-cell lung cancer

AQP4 antibodies and

11.2 322
encephalitis

AQP4 antibodies, area

postrema syndrome, and 11.1 321
lung cancer

Behçet disease and

18.1 479
brainstem encephalitis

Bickerstaff brainstem
13.2 374
encephalitis

CAR T cell neurotoxicity 16.3 448

CASPR2 antibodies and

7.4 201
cerebellar ataxia

CASPR2 antibodies and

24.1 619
dementia

CASPR2 antibodies and

7.5 204
Morvan syndrome

CASPR2 encephalitis
presenting with orthostatic 7.3 201
tremor

cerebral amyloid
angiopathy-related 18.4 492
inflammation

CV2 (CRMP5) antibodies,

12.2 350
cerebellar ataxia, and lung
Topic Number Page

cancer

CV2 (CRMP5) antibodies,

optic neuropathy, and 10.4 307
small-cell lung cancer

Dipeptidyl-peptidase-like
protein 6 (DPPX)
14.3 404
encephalitis, an autopsy
study

DPPX encephalitis, index

5.2 151
case

DPPX encephalitis
24.4 622
mimicking CJD

GABAaR encephalitis and

2.2 28
thymoma

GABAaR encephalitis post-

autologous stem-cell 16.4 450
transplantation

GABAaR encephalitis,
concurrent LGI1 antibodies, 9.2 263
and thymoma

GABAbR encephalitis and

6.5 181
small-cell lung cancer

GABAbR encephalitis
presenting as status 2.1 22
epilepticus

GABAbR encephalitis,
hyponatraemia, and small- 9.1 262
cell lung cancer

GAD antibodies and drug-

23.2 603
resistant epilepsy

GAD antibodies and

paraneoplastic limbic 23.3 604
encephalitis
Topic Number Page

GAD antibodies and stiff-leg

14.1 394
syndrome

GAD antibodies, cerebellar

ataxia, and stiff-person 12.4 354
syndrome

GFAP meningoencephalitis 11.4 332

GlyR antibodies and PERM 14.2 401

Hashimoto encephalopathy
mimicking anti-NMDAR 17.1 464
encephalitis

Hashimoto encephalopathy
19.2 518
presenting as psychosis

Hu (ANNA1) antibodies and

23.1 592
epilepsia partialis continua

Hu (ANNA1) brainstem
encephalitis and small-cell 13.6 381
lung cancer

Hu (ANNA1)
encephalomyelitis and 6.3 178
small-cell lung cancer

Human herpesvirus 6
(HHV-6) as cause of limbic 6.2 175
encephalitis

IgLON5 disease presenting

22.1 580
as a brainstem syndrome

IgLON5 disease presenting

as a progressive
15.2 415
supranuclear palsy-like
syndrome

IgLON5 disease presenting

15.3 416
as chorea

IgLON5 disease presenting

24.3 622
as dementia
Topic Number Page

IgLON5 disease presenting

as motor neuron-like 15.4 416
disorder

IgLON5 disease, sleep

15.1 413
disorder

Immune checkpoint
inhibitors and anti-GABAbR 16.2 445
encephalitis

Immune checkpoint
16.1 443
inhibitors and encephalitis

KLHL11 antibodies,
brainstem encephalitis, and 13.4 378
seminoma

KLHL11 antibodies,
cerebellar ataxia, and 12.3 353
thymic germinoma

LGI1 encephalitis and

faciobrachial dystonic 24.2 621
seizures

LGI1 encephalitis and REM

7.2 197
sleep behaviour disorder

Limbic encephalitis and

17.3 471
TPO antibodies

Limbic encephalitis
seronegative and lung 6.1 171
cancer

Ma2 brainstem encephalitis 13.3 376

Ma2 limbic encephalitis

presenting as isolated 6.4 180
epilepsy

mGluR5 encephalitis and

6.6 182
Hodgkin disease

MOG antibodies and ADEM 10.1 295

Topic Number Page

(acute disseminated
encephalomyelitis)

MOG antibodies and

10.2 302
brainstem encephalitis

MOG antibodies and

10.3 303
cortical encephalitis

Myelopathy, seronegative,
11.3 326
and breast cancer

Neuromyotonia and small-

21.4 555
cell lung cancer

NMDAR antibodies in
choreoathetosis post- 21.1 547
herpes simplex encephalitis

NMDAR encephalitis and

intracranial hypertension, 8.3 214
an early case

NMDAR encephalitis
misdiagnosed as 17.2 466
Hashimoto encephalopathy

NMDAR encephalitis
misdiagnosed as
8.2 213
neuroleptic malignant
syndrome

NMDAR encephalitis
1.1 7
misdiagnosed as PANDAS

NMDAR encephalitis, a
complicated course in a 20.1 529
child

NMDAR encephalitis, index

8.1 212
case

NMDAR encephalitis,
8.4 224
psychiatric presentation

NMDAR encephalitis, the 20.2 536

Topic Number Page

difficulty of determining
symptomatic treatment
efficacy

Opsoclonus-myoclonus
syndrome and breast 13.1 372
cancer

PANDAS 19.1 516

Primary angiitis of the CNS

18.3 487
(PACNS)

Ri (ANNA2) brainstem
encephalitis and breast 13.5 379
cancer

Ri (ANNA2) parkinsonism
21.2 551
and breast cancer

Sarcoidosis and
18.2 484
encephalitis

Susac syndrome 18.5 494

VGKC antibodies leading to

7.1 193
misdiagnosis of glioma

Yo (PCA1) antibodies,
cerebellar ataxia, and 12.1 349
breast cancer
Videos
Published online by Cambridge University Press: 27 January 2022

Josep Dalmau and

Francesc Graus

(In alphabetical order of the antibody or neurological syndrome)

Topic Number Page
AMPAR encephalitis 20.3 539
Amphiphysin antibodies and spinal 21.7 554
myoclonus
CASPR2 antibodies and episodic 21.9 556
dysarthria
CASPR 2 antibodies and orthostatic 21.5 553
myoclonus
CASPR 2 antibodies, Morvan syndrome, 22.1 572
and agrypnia excitata
CV2 (CRMP5) antibodies and chorea 21.2 549
GABAbR encephalitis and seizures 2.1 22
GAD antibodies and stiff-leg syndrome 14.1 394
Hashimoto encephalopathy 17.1 464
Hu antibodies and epilepsia partialis 23.1 592
continua
lgl_ON5 disease and chorea 15.3 416
lgl_ON5 disease and facial myokymias 21.3 551
and myorhythmia
lgl_ON5 disease and NREM parasomnia 15.1 413
lgl_ON5 disease and NREM 22.2 576
parasomnias despite CPAP treatment
lgLON5 disease, gait instability, and 15.2 415
abnormal postural reflexes
LGI 1 antibodies and faciobrachial 9.1 272
dystonic seizures
LGI 1 encephalitis and chorea 7.1 197
LGI 1 encephalitis and memory 7.2 197
deficit
Topic Number Page

AMPAR encephalitis 20.3 539

Amphiphysin antibodies
21.7 554
and spinal myoclonus

CASPR2 antibodies and

21.9 556
episodic dysarthria

CASPR2 antibodies and

21.5 553
orthostatic myoclonus

CASPR2 antibodies, Morvan

syndrome, and agrypnia 22.1 572
excitata

CV2 (CRMP5) antibodies

21.2 549
and chorea

GABAbR encephalitis and

2.1 22
seizures

GAD antibodies and stiff-leg

14.1 394
syndrome

Hashimoto encephalopathy 17.1 464

Hu antibodies and epilepsia

23.1 592
partialis continua

IgLON5 disease and chorea 15.3 416

IgLON5 disease and facial

myokymias and 21.3 551
myorhythmia

IgLON5 disease and NREM

15.1 413
parasomnia

IgLON5 disease and NREM

parasomnias despite CPAP 22.2 576
treatment

IgLON5 disease, gait

instability, and abnormal 15.2 415
postural reflexes

LGI1 antibodies and 9.1 272

Topic Number Page

faciobrachial dystonic
seizures

LGI1 encephalitis and

7.1 197
chorea

LGI1 encephalitis and

7.2 197
memory deficit

LGI1 encephalitis mimicking

24.1 621
CJD

Ma2 encephalitis and ocular

13.2 376
findings

Neuromyotonia and small-

21.8 555
cell lung cancer

NMDAR antibodies and

choreoathetosis post- 21.1 547
herpes simplex encephalitis

NMDAR encephalitis and

8.3 226
cataleptic postures

NMDAR encephalitis and

20.2 530
catatonia

NMDAR encephalitis and

8.1 226
orofacial dyskinesias

NMDAR encephalitis and

pseudo piano-playing 8.2 226
stereotypies

NMDAR encephalitis and

20.1 529
psychosis

Opsoclonus 13.1 369

PERM (progressive
encephalomyelitis with
21.6 553
rigidity and myoclonus) and
hyperekplexia

Ri (ANNA2) antibodies and 21.4 551

Topic Number Page

parkinsonism

Rotarod test 5.1 156

Preface
Published online by Cambridge University Press: 27 January 2022

Josep Dalmau and

Francesc Graus

Dante Alighieri, Inferno, canto 1, The divine comedy.

Why did we write this book? The field of autoimmune encephalitis and related disorders
of the nervous system has grown exponentially in the last decade with the identification
of disorders that share two important features: they often have a subacute clinical onset
that in a few weeks transports a person from an independent, healthy life to one of
functional impairment with severe neurological deficits. The other feature is that prompt
diagnosis and treatment reverse this downhill clinical course and frequently result in
good outcomes. Similar to what Dante graphically depicted in the first verses of the
Divine Comedy, the patient with autoimmune encephalitis is all of a sudden in a
shadowed forest, lost on a path that drives to darkness. In this situation, no matter how
sophisticated our technology, how many antibodies are detected or clinical criteria
fulfilled, and how good the healthcare system might be, the key issue is whether the
treating physician has sufficient knowledge of these diseases and their pathogenic
mechanisms to guide clinical reasoning that will lead to optimal outcomes. We wrote this
book with this in mind and have tried to condensate the knowledge we believe may help
to make the right decisions guiding, as Virgil did with Dante, the patient out of darkness.

The book has 25 chapters divided into four sections. In the first section we offer a
historical overview on how the discovery of antibody-mediated diseases of the peripheral
nervous system and paraneoplastic neurological syndromes paved the way for the
characterization of autoimmune encephalitides. We also provide general diagnostic
guidelines, and review the pathophysiological mechanisms involved in the different types
of autoimmune encephalitis. The second section focuses on the characteristics of
neuronal and glial antibodies that we have divided according to the cellular location of
the antigen: intracellular or cell surface. The third section describes specific types of
autoimmune encephalitis according to the type of antibodies (e.g., anti-NMDAR
encephalitis) or anatomical regions involved, such as the limbic system, cerebellum,
brainstem, or the spinal cord. In this section we also include neurological disorders
typically classified as demyelinating diseases, such as MOG antibody-associated disease,
which may overlap with autoimmune encephalitis, and another group of autoimmune
syndromes which we have called ‘frontier disorders’, that are frequently considered in
the differential diagnosis of autoimmune encephalitis. In the fourth section we address
the relevance of autoimmune mechanisms in some subspecialty areas within neurology
and psychiatry, such as sleep, movement disorders, or psychosis, and provide a critical
review of concepts such as autoimmune psychosis, autoimmune epilepsy, and
autoimmune dementia that we believe do not add clinical or diagnostic value to that of
autoimmune encephalitis. In the last chapter we provide a list of relevant questions that
many colleagues have asked us over the years. Our answers summarize some of the
information and concepts developed in the previous chapters and we hope readers will
find them useful.

Some books, like some people, do not age well as the ideas provided may quickly become
outdated. This possibility is unlikely to occur with this book. It has been written when the
field of autoimmune encephalitis has reached maturity and the most important concepts
have been defined. New advances will complement the information and ideas contained
here, but the fundamental concepts will likely not change. Even though this book
represents the opinions of the two authors, some chapters were critically reviewed by
colleagues who provided inestimable comments. They are acknowledged at the ends of
the chapters along with colleagues who provided clinical information and figures for the
vignettes. Here we would like to give special thanks to Dr Myrna R. Rosenfeld, who
reviewed all the chapters, Dr Jesus Planagumà for his help in the design and
development of some of the figures, and Dr Ellen Gelpi for providing almost all
neuropathological figures.

Finally, the writing of this book and the approach to selected topics and clinical questions
were inspired by the teachings and example of Dr Jerome B. Posner. Without his
mentorship this book would never have been written. He guided our first steps in the
field of paraneoplastic neurological syndromes and showed us how to formulate clinical
questions before addressing them in the lab. We have tried to apply this guidance here,
and any problems or limitations that the reader may find are ours.

This book provides access to an online version on Cambridge Core, which can be
accessed via the code printed on the inside of the cover.
Abbreviations
Published online by Cambridge University Press: 27 January 2022

Josep Dalmau and

Francesc Graus

Abbreviations
Antibodies
AChR
acetylcholine receptor

AK5
adenylate kinase 5

AMPAR
α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor

AQP4
aquaporin 4

CASPR2
contactin-associated protein-like 2

CRMP5
collapsing response-mediator protein 5

DNER
Delta/Notch-like epidermal growth factor-related receptor

DPPX
dipeptidyl-peptidase-like protein 6
GABAR
gamma-aminobutyric acid receptor

GAD
glutamic acid decarboxylase

GFAP
glial fibrillary acidic protein

GluK2
glutamate kainate receptor subunit 2

GlyR
glycine receptor

KLHL11
Kelch-like protein 11

LGI1
leucine-rich, glioma inactivated 1

MAP1B
microtubule-associated protein 1B

mGluR
metabotropic glutamate receptor

MOG
myelin oligodendrocyte glycoprotein

NMDAR
N-methyl-D-aspartate receptor
TPO
thyroid peroxidase

VGCC
voltage-gated calcium channel

VGKC
voltage-gated potassium channel

Other Abbreviations
ADEM
acute disseminated encephalomyelitis

AHSCT
autologous haematopoietic stem-cell transplantation

ASM
anti-seizure medication

BBE
Bickerstaff brainstem encephalitis

CARs
chimeric antigen receptors

CBA
cell-based assay

CDR
cerebellar degeneration-related

CJD
Creutzfeldt–Jakob disease
CLIPPERS
chronic lymphocytic inflammation with pontine perivascular enhancement responsive to
steroids

CNS
central nervous system

CPAP
continuous positive airway pressure

CRS
cytokine-release syndrome

CSF
cerebrospinal fluid

CTLA-4
cytotoxic T lymphocyte-associated protein 4

DM1
diabetes mellitus type 1

EBV
Epstein–Barr virus

EEG
electroencephalogram

ECT
electroconvulsive therapy

EDB
extreme delta brush
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A FEW GENERAL RULES AND DIRECTIONS FOR PRESERVING.

1. Let everything used for the purpose be delicately clean and dry;
bottles especially so.
2. Never place a preserving-pan flat upon the fire, as this will
render the preserve liable to burn to, as it is called; that is to say, to
adhere closely to the metal, and then to burn; it should rest always
on a trivet (that shown with the French furnace is very convenient
even for a common grate), or on the lowered bar of a kitchen range
when there is no regular preserving stove in a house.
3. After the sugar is added to them, stir the preserves gently at
first, and more quickly towards the end, without quitting them until
they are done: this precaution will always prevent the chance of their
being spoiled.
4. All preserves should be perfectly cleared from the scum as it
rises.
5. Fruit which is to be preserved in syrup must first be blanched or
boiled gently, until it is sufficiently softened to absorb the sugar; and
a thin syrup must be poured on it at first, or it will shrivel instead of
remaining plump, and becoming clear. Thus, if its weight of sugar is
to be allowed, and boiled to a syrup with a pint of water to the pound,
only half the weight must be taken at first, and this must not be
boiled with the water more than fifteen or twenty minutes at the
commencement of the process; a part of the remaining sugar must
be added every time the syrup is reboiled, unless it should be
otherwise directed in the receipt.
6. To preserve both the true flavour and the colour of fruit in jams
and jellies, boil them rapidly until they are well reduced, before the
sugar is added, and quickly afterwards, but do not allow them to
become so much thickened that the sugar will not dissolve in them
easily, and throw up its scum. In some seasons, the juice is so much
richer than in others, that this effect takes place almost before one is
aware of it; but the drop which adheres to the skimmer when it is
held up, will show the state it has reached.
7. Never use tin, iron, or pewter spoons, or skimmers, for
preserves, as they will convert the colour of red fruit into a dingy
purple, and impart, besides, a very unpleasant flavour.
8. When cheap jams or jellies are required, make them at once
with Lisbon sugar, but use that which is well refined always, for
preserves in general; it is a false economy, as we have elsewhere
observed, to purchase an inferior kind, as there is great waste from it
in the quantity of scum which it throws up. The best has been used
for all the receipts given here.
9. Let fruit for preserving be gathered always in perfectly dry
weather, and be free both from the morning and evening dew, and as
much so as possible from dust. When bottled, it must be steamed or
baked during the day on which it is gathered, or there will be a great
loss from the bursting of the bottles; and for jams and jellies it cannot
be too soon boiled down after it is taken from the trees.
 TO EXTRACT THE JUICE OF PLUMS FOR JELLY.

Take the stalks from the fruit, and throw aside all that is not
perfectly sound: put it into very clean, large stone jars, and give part
of the harder kinds, such as bullaces and damson, a gash with a
knife as they are thrown in; do this especially in filling the upper part
of the jars. Tie one or two folds of thick paper over them, and set
them for the night into an oven from which the bread has been drawn
four or five hours; or cover them with bladder, instead of paper, place
them in pans, or in a copper[166] with water which will reach to quite
two-thirds of their height, and boil them gently from two to three
hours, or until the fruit is quite soft, and has yielded all the juice it will
afford: this last is the safer and better mode for jellies of delicate
colour.
166. The fruit steams perfectly in this, if the cover be placed over.
 TO WEIGH THE JUICE OF FRUIT.

Put a basin into one scale, and its weight into the other; add to this
last the weight which is required of the juice, and pour into the basin
as much as will balance the scales. It is always better to weigh than
to measure the juice for preserving, as it can generally be done with
more exactness.
 RHUBARB JAM.

The stalks of the rhubarb (or spring-fruit, as it is called) should be

taken for this preserve, which is a very good and useful one, while
they are fresh and young. Wipe them very clean, pare them quickly,
weigh, and cut them into half-inch lengths; to every pound add an
equal weight of good sugar in fine powder; mix them well together,
let them remain for ten minutes or a quarter of an hour to draw out
the juice a little, then turn them into a preserving-pan, let them heat
rather slowly, but as soon as the stalks are tender boil the preserve
rapidly, stirring it well for about half an hour. It will be of excellent
flavour, and will serve admirably for tarts.
A somewhat cheaper mode of making the jam is to stew it until
tender in its own juices, and then to boil it rapidly until it is tolerably
dry, to add to it only half its weight of sugar, and to give it from twenty
to thirty minutes boiling.
Spring fruit (rhubarb), 4 lbs.; sugar, 4 lbs.: heated slowly, and
when tender, boiled quickly, 30 minutes.
 GREEN GOOSEBERRY JELLY.

Wash some freshly gathered gooseberries very clean; after having

taken off the tops and stalks, then to each pound pour three-quarters
of a pint of spring water, and simmer them until they are well broken;
turn the whole into a jelly-bag or cloth, and let all the juice drain
through; weigh and boil it rapidly for fifteen minutes. Draw it from the
fire, and stir in it until entirely dissolved, an equal weight of good
sugar reduced to powder; boil the jelly from fifteen to twenty minutes
longer, or until it jellies strongly on the spoon or skimmer; clear it
perfectly from scum, and pour it into small jars, moulds, or glasses. It
ought to be very pale and transparent. The sugar may be added to
the juice at first, and the preserve boiled from twenty-five to thirty-five
minutes, but the colour will not then be so good. When the fruit
abounds, the juice may be drawn from it with very little water, as
directed for apples, page 523, when it will require much less boiling.
Gooseberries, 6 lbs.; water, 4 pints: 20 to 30 minutes. Juice boiled
quickly, 15 minutes; to each pound, 1 pound sugar: 15 to 20 minutes.
 GREEN GOOSEBERRY JAM.

(Firm and of good colour.)

Cut the stalks and tops from the fruit, weigh and bruise it slightly,
boil it for six or seven minutes, keeping it well turned during the time,
then to every three pounds of gooseberries add two and a half of
sugar beaten to powder, and boil the preserve quickly for three-
quarters of an hour. It must be constantly stirred, and carefully
cleared from scum. This makes a fine, firm, and refreshing preserve
if the fruit be rubbed through a sieve before the sugar is added. If
well reduced afterwards, it may be converted into a gâteau, or
gooseberry-solid, with three pounds of sugar, or even a smaller
proportion. The preceding jam will often turn in perfect form from the
moulds or jars which contain it; and if freed from the seeds, would be
very excellent: it is extremely good even made as above. For all
preserves, the reduction, or boiling down to a certain consistence,
should take place principally before the sugar is mingled with them;
and this has the best effect when added to the fruit and dissolved in
it by degrees.
Green gooseberries, 6 lbs.: 6 to 7 minutes. Sugar, 5 lbs.; 3/4 hour.
 TO DRY GREEN GOOSEBERRIES.

Take the finest green gooseberries, fully grown, and freshly

gathered; cut off the buds, split them across the tops half way down,
and with the small end of a tea or of an egg spoon, scoop out the
seeds. Boil together for fifteen minutes a pound and a half of the
finest sugar, and a pint of water; skim this syrup thoroughly and
throw into it a pound of the seeded gooseberries; simmer them from
five to seven minutes, when they ought to be clear and tender; when
they are so, lift them out, and throw as many more into the syrup;
drain them a little when done, spread them singly on dishes, and dry
them very gradually in a quite cool stove or oven, or in a sunny
window. They will keep well in the syrup, and may be potted in it, and
dried when wanted for use.
Green gooseberries without seeds, 2 lbs.; water, 1 pint; sugar, 1-
1/2 lb.: boiled, 15 minutes. Gooseberries simmered, 5 to 7 minutes.
 GREEN GOOSEBERRIES FOR TARTS.

Fill very clean, dry, wide-necked bottles with gooseberries

gathered the same day, and before they have attained their full
growth. Cork them lightly, wrap a little hay round each of them, and
set them up to their necks in a copper of cold water which should be
brought very gradually to boil. Let the fruit be gently simmered until it
appears shrunken and perfectly scalded; then take out the bottles,
and with the contents of one or two fill up the remainder, and use
great care not to break the fruit in doing this. When all are ready pour
scalding water into the bottles and cover the gooseberries entirely
with it, or they will become mouldy at the top. Cork the bottles well
immediately, and cover the necks with melted resin; keep them in a
cool place; and when the gooseberries are used pour off the greater
part of the water, and add sugar as for the fresh fruit, of which they
will have the flavour and appearance; and they will be found more
wholesome prepared in this manner than if simply baked or steamed
in the bottles.
 RED GOOSEBERRY JAM.

The small rough red gooseberry, when fully ripe, is the best for this
preserve, which may, however, be made of the larger kinds. When
the tops and stalks have been taken carefully from the fruit, weigh,
and boil it quickly for three-quarters of an hour, keeping it well stirred;
then for six pounds of the gooseberries, add two and a half of good
roughly-powdered sugar; boil these together briskly, from twenty to
twenty-five minutes and stir the jam well from the bottom of the pan,
as it is liable to burn if this be neglected.
Small red gooseberries, 6 lbs.: 3/4 hour. Pounded sugar, 2-1/2
lbs.: 20 to 25 minutes.
 VERY FINE GOOSEBERRY JAM.

Seed the fruit, which for this jam may be of the larger kind of rough
red gooseberry: those which are smooth skinned are generally of far
inferior flavour. Add the pulp which has been scooped from the
prepared fruit to some whole gooseberries, and stir them over a
moderate fire for some minutes to extract the juice; strain and weigh
this; pour two pounds of it to four of the seeded gooseberries, boil
them rather gently for twenty-five minutes, add fourteen ounces of
good pounded sugar to each pound of fruit and juice, and when it is
dissolved boil the preserve from twelve to fifteen minutes longer, and
skim it well during the time.
Seeded gooseberries, 4 lbs.; juice of gooseberries, 2 lbs.: 25
minutes. Sugar, 5-1/4 lbs. (or 14 oz. to each pound of fruit and juice):
12 to 15 minutes.
 JELLY OF RIPE GOOSEBERRIES.

(Excellent.)
Take the tops and stalks from a gallon or more of any kind of well-
flavoured ripe red gooseberries, and keep them stirred gently over a
clear fire until they have yielded all their juice, which should then be
poured off without pressing the fruit, and passed first through a fine
sieve, and afterwards through a double muslin-strainer, or a jelly-
bag. Next weigh it, and to every three pounds add one of white
currant juice, which has previously been prepared in the same way;
boil these quickly for a quarter of an hour, then draw them from the
fire and stir to them half their weight of good sugar; when this is
dissolved, boil the jelly for six minutes longer, skim it thoroughly, and
pour it into jars or moulds. If a very large quantity be made, a few
minutes of additional boiling must be given to it before the sugar is
added.
Juice of red gooseberries, 3 lbs.; juice of white currants, 1 lb.: 15
minutes. Sugar, 2 lbs.: 6 minutes.
Obs.—The same proportion of red currant juice, mixed with that of
the gooseberries, makes an exceedingly nice jelly.
 UNMIXED GOOSEBERRY JELLY.

Boil rapidly for ten minutes four pounds of the juice of red
gooseberries, prepared as in the preceding receipt; take it from the
fire, and stir in it until dissolved three pounds of sugar beaten to
powder; boil it again for five minutes, keeping it constantly stirred
and thoroughly skimmed.
Juice of red gooseberries, 4 lbs.: 10 minutes. Sugar, 3 lbs.: 5
minutes.
 GOOSEBERRY PASTE.

Press through a sieve the gooseberries from which the juice has
been taken for jelly, without having been drained very closely from
them; weigh and then boil the pulp for upwards of an hour and a
quarter, or until it forms a dry paste in the pan; stir to it, off the fire,
six ounces of good pounded sugar for each pound of the fruit, and
when this is nearly dissolved boil the preserve from twenty to twenty-
five minutes, keeping it stirred without cessation, as it will be liable to
burn should this be neglected. Put it into moulds, or shallow pans,
and turn it out when wanted for table.
Pulp of gooseberries, 4 lbs.: 1-1/4 to 1-3/4 hour. Sugar, 1-1/2 lb.:
20 to 25 minutes.
 TO DRY RIPE GOOSEBERRIES WITH SUGAR.

Cut the tops, but not the stalks, from some ripe gooseberries of
the largest size, either red or green ones, and after having taken out
the seeds as directed for unripe gooseberries, boil the fruit until clear
and tender, in syrup made with a pound of sugar to the pint of water,
boiled until rather thick.
Seeded gooseberries, 2 lbs.; sugar, 1-1/2 lb.; water, 1 pint: boiled
to syrup. Gooseberries, simmered 8 to 12 minutes, or more.
Obs.—Large ripe gooseberries freed from the blossoms, and put
into cold syrup in which cherries or any other fruit has been boiled for
drying, then heated very gradually, and kept at the point of boiling for
a few minutes before they are set by for a couple of days, answer
extremely well as a dry preserve. On the third day the syrup should
be drained from them, simmered, skimmed, and poured on them the
instant it is taken from the fire; in forty-eight hours after, they may be
drained from it and laid singly upon plates or dishes, and placed in a
gentle stove.
 JAM OF KENTISH OR FLEMISH CHERRIES.

This is a very agreeable preserve when it is made as we shall

direct; but if long boiled with a large proportion of sugar, as it
frequently is, both the bright colour and the pleasant flavour of the
cherries will be destroyed.
Stone, and then weigh the fruit; heat it rather slowly that the juice
may be well drawn out before it begins to boil, and stew the cherries
until they are tolerably tender, then boil them quickly, keeping them
well turned and stirred from the bottom of the pan, for three-quarters
of an hour or somewhat longer should there still remain a large
quantity of juice. Draw the pan from the fire, and stir in gradually half
a pound of sugar for each pound of cherries. An ounce or two more
may occasionally be required when the fruit is more than usually
acid, and also when a quite sweet preserve is liked. When the sugar
is dissolved continue the boiling rapidly for about twenty minutes
longer; clear off all the scum as it appears, and keep the jam stirred
well and constantly, but not quickly, to prevent its adhering to the
bottom of the preserving-pan.
Stoned Kentish or Flemish cherries, 6 lbs.: without sugar, 1 hour or
rather more. Sugar roughly powdered, 3 lbs.: (or 3-1/2 lbs.) About 20
minutes quick boiling.
Obs.—Heat the fruit and boil it gently until it is quite tender, turning
it often, and pressing it down into the juice; then quicken the boiling
to evaporate the juice before the sugar is added. Cherries which are
bruised will not make good preserve: they always remain tough.
 TO DRY CHERRIES WITH SUGAR.

(A quick and easy method.)

Stone some fine, sound, Kentish or Flemish cherries; put them into
a preserving-pan, with six ounces of sugar reduced to powder, to
each pound of the fruit; set them over a moderate fire, and simmer
them gently for nearly or quite twenty minutes; let them remain in the
syrup until they are a little cooled, then turn them into a sieve, and
before they are cold lay them singly on dishes, and dry them very
gradually, as directed for other fruits. When the cherries are quite
ripe the stones may generally be drawn out with the stalks, by
pressing the fruit gently at the same time; but when this method fails,
they must be extracted with a new quill, cut round at the end: those
of the very short-stalked, turnip-shaped cherry, which abounds, and
is remarkably fine in many parts of Normandy, and which we have
occasionally met with here, though it is not, we believe, very
abundant in our markets, are easily removed with a large pin, on the
point of which the stone may be caught at the stalk end, just
opposite the seam of the fruit, and drawn out at the top, leaving the
cherry apparently entire.
 DRIED CHERRIES.

(Superior Receipt.)
To each pound of cherries weighed after they are stoned, add
eight ounces of good sugar, and boil them very softly for ten minutes:
pour them into a large bowl or pan, and leave them for two days in
the syrup; then simmer them again for ten minutes, and set them by
in it for two or three days; drain them slightly, and dry them very
slowly, as directed in the previous receipts. Keep them in jars or tin
canisters, when done. These cherries are generally preferred to such
as are dried with a larger proportion of sugar; but when the taste is in
favour of the latter, from twelve to sixteen ounces can be allowed to
the pound of fruit, which may then be potted in the syrup and dried at
any time; though we think the flavour of the cherries is better
preserved when this is done within a fortnight of their being boiled.
Cherries, stoned, 8 lbs.; sugar, 4 lbs.: 10 minutes. Left two or three
days. Boiled again, 10 minutes; left two days; drained and dried.
 CHERRIES DRIED WITHOUT SUGAR.

These are often more pleasant and refreshing to invalids and

travellers than a sweetened confection of the fruit, their flavour and
agreeable acidity being well preserved when they are simply spread
on dishes or hamper-lids, and slowly dried.[167] Throw aside the
bruised and decayed fruit, and arrange the remainder singly, and
with the stalks uppermost on the dishes. The Kentish cherries are
best for the purpose, but morellas also answer for it excellently. The
former are sometimes stoned, and simmered until quite tender in
their own juice, before they are dried; but this is scarcely an
improvement on the more usual method of leaving them entire.
167. The dishes on which they are laid should be changed daily.
 TO DRY MORELLA CHERRIES.

Take off the stalks but do not stone the fruit; weigh and add to it an
equal quantity of the best sugar reduced quite to powder, strew it
over the cherries and let them stand for half an hour; then turn them
gently into a preserving-pan, and simmer them softly from five to
seven minutes. Drain them from the syrup, and dry them like the
Kentish cherries. They make a very fine confection.