Clinical Neurophysiology 141 (2022) S141–S185
c
CHUV - Centre hospitalier universitaire vaudois, Lausanne, Switzerland
d
Universitats Kinderspital Zurich, Zurich, Switzerland
e
EOC - Ente Ospedaliero Cantonale, Bellinzona, Switzerland
Introduction: Epilepsy with myoclonic-atonic seizures (EMAtS), for-
merly known as Doose syndrome, is defined as a generalized epi-
lepsy syndrome characterized by the onset of different seizure
types between 6 months and 6 years, in previously healthy children.
The outcome for children affected by EMAtS is variable, from com-
plete remission and normal development to therapy resistant epi-
lepsy and intellectual disability. Currently, it is not possible to
predict early in the course if a child diagnosed with EMAtS will
evolve positively or negatively. This retrospective study aims to
assess the prognostic value of different EEG patterns on traces regis-
tered in the first year after seizure onset in these children.
Methods: A retrospective multicentric study was conducted in
Switzerland, including the neuropediatric units of Geneva, Basel,
Bellinzona, Lausanne, and Zurich. Subjects were recruited among
patients who received a diagnosis of EMAtS and for whom EEG
recordings during the first year after seizure onset were available.
A total of 47 patients were included. Two groups were created
according to cognitive outcome (group A with favorable outcome
defined as normal schooling; group B with poor outcome, defined
as special schooling).
Clinical parameters included patient demographic data, family his-
tory of seizures, age of onset and type of seizures, treatments used
and their sequence of introduction, duration of the epilepsy, seizure
freedom, EEG features and school course. EEG interpretation was
based on the analysis of all EEGs recorded during the first year after
seizure onset. Each EEG was read by two experienced clinicians.
Results: No significant differences were identified among EEG data
of the two groups regarding background activity, structure of sleep,
presence of focal anomalies, slow delta or theta activity. Seizure
types during the first year do not seem to be a marker of outcome,
either, although isolated atonic seizures were more frequent in
group B. Global prognosis proved to be most favorable among
patients who showed a higher IQ/DQ during the early stages of the
disease.
Conclusions: Our findings suggest that no specific EEG feature can
be considered as reliable predictor for outcome in patients with
EMAtS. Major strengths of this study are the blinding of the EEG
readers to outcome and the long patient follow up. Our study has
several limitations, which include its small sample size and retro-
spective design. Further studies with a larger sample size and a
prospective design are required to unveil the underlying mecha-
nisms associated to outcome.
doi:10.1016/j.clinph.2022.07.443
TH-258. Isolated nuclear damage of the musculocutaneous nerve
Khalida Mammadova *, Abir Alaamel, Hilmi Uysal
Akdeniz University Department of Neurology, Antalya, Turkey
Introduction: Isolated brachial muscle atrophy is a rare pathology.
There are limited number of cases in the literature. We present a
case who presented with a brachial muscle atrophy without any eti-
ological risk factor.
Methods and Results: A 55-year-old male patient applied to the
neurology outpatient clinic with the complaint of weakness in the
right arm. He had a complaint for 3 months. His weakness increased
especially when he was tired. On neurological examination, he is
conscious and oriented. Muscle strength examination was normal.
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Deep tendon reflexes were normoactive in both upper and lower
extremities. Sensory examination was normal. Measurement made
20 cm distal from the shoulder showed that the right arm circumfer-
ence was 31 cm and the left arm circumference was 33 cm.
Measurement made 14 cm distal from the elbow showed that the
right arm circumference was 25 cm and the left arm circumference
was 25.5 cm. In EMG, there were findings consistent with chronic
partial degeneration and regeneration of the branch of the musculo-
cutaneous nerve to the brachialis muscle on the right. There was no
pathology to explain in the cervical MRI. We believe that this finding
may be an indicator of nucleus damage of the musculocutaneous
nerve branch to the brachialis muscle.
Conclusion: In the literature, there are cases reported after heavy
lifting or trauma. Karl et al. reported isolated brachial muscle atro-
phy after heavy lifting. We could not detect any risk factors in our
case.
doi:10.1016/j.clinph.2022.07.444
TH-259. Atypical chronic inflammatory demyelinating
polyneuropathy cases, focal CIDP
Hilmi Uysal a,*, Gürsel Leblebicioğlu b, Levent Albayrak c, Esra
Asßıkdoğan a, Abir Alaamel a, Sultan Didem Aydeniz a, Mehmet
Yörebulut d, Kamil Karaali e
a
Akdeniz University Neurology Department, Antalya, Turkey
b
Hacettepe University Ortopedics and Traumatology Department Ortopedics and Trau-
matology Department Ortopedics and Traumatology, Ankara, Turkey
c
Bayırdır Söğütözü Hospital Pathology Department, Ankara, Turkey
d _
Acıbadem Hospital Radiology Department, Istanbul, Turkey
e
Akdeniz University Radiology Department, Antalya, Turkey
Introduction: CIDP is a rare neuropathy with typical, atypical forms.
In 2021, EAN’s guidelines distal, multifocal, focal, motor, sensory
forms are defined. Focal CIDP is described motor and sensorimotor
neuropathy confined to one limb. This abstract includes cases which
presented focal CIDP’s radiological, pathological, electrophysiological
findings:
 22-year-old woman, complaint left arm weakness, atrophy. In
examination proximal muscle power 3/5 was detected, biceps,
triceps were absent. In laboratory, immune electrophoresis,
hba1c, vasculitis, infections, thyroid test (out of TRAb) were
detected normal. High TRAb was not found significant by
endocrinology. SSEP, MEP were normal. In EMG, neurogenic
MUAP were seen muscles which were stimulated upper, middle
truncus. In MRI, C4-T1 roots were detected thicker than normal,
contrasty. Malignancy was not detected in biopsy.
Corticosteroid was practiced. Because of MRI continuation, IVIG
was practiced, patient use IVIG once in a month.
 20-year-old man, complaint left arm weakness. In examination,
distal weakness, interossei muscle atrophy were detected. In lab-
oratory, immune electrophoresis, vasculitis (out of LAC), infec-
tions, hba1c, IGG4, angiotensin converting enzyme, lumbar
punction and NF 155, thyroid tests (out of TSH, TRab, Anti-
HTG), were detected normal. LAC and thyroid test were not found
significant. SSEP, MEP normal. In EMG neurogenic MUAP were
seen distal muscles. In MRI, left plexus supraclavicular division
was detected thicker than normal, contrasty. Corticosteroid was
practiced. Because of MRI continuation, IVIG treatment was prac-
ticed, patient use IVIG once in a month.
 20-year-old man, complaint left arm weakness. In examination,
distal weakness, hypotenar, tenar atrophy were detected, upper
extremity reflexes were absent. In laboratory, immune
 Clinical Neurophysiology 141 (2022) S141–S185
electrophoresis, vasculitis, infections, hba1c, angiotensin convert-
ing enzyme, thyroid test, lumbar punction were detected normal.
In EMG neurogenic MUAP were seen muscles which were stimu-
lated all truncus of plexus. In MRI, left brachial plexus all fibers
were detected thicker than normal, contrasty. Malignancy was
not detected in biopsy. Patient use corticosteroid.
Discussion: Focal CIDP is one of the atypical forms of CIDP. In 1996,
Thomas and friends published case serial about focal CIDP. Serial
included patients who had only motor, sensorial or sensorimotor
demyelinating neuropathy in one of the upper extremities. When
patients are evaluated for focal CIDP; diabetic polyneuropathy, mul-
tifocal motor neuropathy, hereditary pressure sensitive neuropathy,
neuralgic amyotrophy, peripheral nerve sheath tumors, vasculitis
neuropathies are excluded. At least of two nerve in one extremity
possess motor and sensorial conduction criteria in electrophysiolog-
ical assessment. In brachial, lumbosacral plexus MRI, signal increas-
ing root hypertrophy, contrasty nerve can be observed.
Conclusion: In this abstract, three patients are presented with their
clinic, radiological, electrophysiological, laboratory parameters.
Vasculitis, infection, infiltration, connective tissue disease are
excluded in these patients. When findings are evaluated together,
these patients are considered compatible with focal CIDP.
doi:10.1016/j.clinph.2022.07.445
TH-260. Clinical and electrophysiological features of idiopathic
chronic immune sensory polyneuropathy
Yeon Hak Chung *, Hyunjin Ju, Byoung Joon Kim
Department of Neurology, Samsung Medical Center, Sungkyunkwan University School of
Medicine, Seoul, Republic of Korea
Chronic sensory polyneuropathy has various causes that are difficult
to differentiate in clinical practice. It may be classified as chronic
immune sensory polyradiculopathy (CISP) or pure sensory chronic
inflammatory demyelinating polyradiculoneuropathy (CIDP) when
there are no specific causes such as systemic diseases and responds
to immunotherapy. European Academy of Neurology/Peripheral
Nerve Society (EAN/PNS) guidelines recommended sensory nerve
conduction criteria for the diagnosis of possible sensory CIDP, but
there are still gray areas in the classification of idiopathic chronic
immune sensory polyneuropathies.
We retrospectively analyzed medical data from 10 patients with
chronic progressive pure sensory polyneuropathy who responded
to immunomodulatory treatment. All patients had normal motor
nerve conduction parameters and no possible causes associated with
peripheral neuropathies such as paraneoplastic syndrome, multiple
myeloma, anti-MAG antibody or systemic vasculitis. Clinical mani-
festations, responses to treatment, nerve conduction studies (NCS),
and sural nerve pathology were analyzed.
Age of onset of the patients were 47.0±16.2 years old and seven men
and three women were included. The median disease duration to the
time of NCS was 17.5 months. Prominent and disabling clinical fea-
tures were sensory ataxia (90%) and pain (80%). Other clinical and
laboratory findings were generalized areflexia (80%), elevation of
CSF protein (50%), response to treatment (100%, responses by defini-
tion were evaluated by changes of modified Rankin scale or numer-
ical rating scale after treatment such as steroid pulse,
immunoglobulin, or immunosuppressant), and demyelinating or
mixed feature in 7/8 nerve biopsies (86%). None of the patients
met the sensory nerve conduction criteria of EAN/PNS guideline.
Clinical manifestations in our patients were similar to CISP but NCS
and sural nerve pathology indicated postganglionic sensory axon/
S169
myelin involvement. On the other hand, NCS did not meet the sen-
sory nerve conduction criteria of CIDP. For early initiation of
immunotherapy, it would be helpful to develop more sensitive diag-
nostic criteria and practical classification of chronic immune sensory
polyneuropathy.
doi:10.1016/j.clinph.2022.07.446
TH-261. A case of GBS-like polyneuropathy with unusual pro-
gression (Case Report)
⇑
Hnin Hay Mar a, , Maria Borghi a, Wint Nandar Hein a, Santiago
Catania a, Michael Lunn b
a
Department of Clinical Neurophysiology, National Hospital for Neurology and Neuro-
surgery, London, United Kingdom
b
Centre for Neuromuscular Disease, National Hospital for Neurology and Neurosurgery,
London, United Kingdom
Nodal and paranodal antibody-associated neuropathies have been
recently classified as auto-immune nodopathies. Their clinical pre-
sentation can be easily mistaken for acute or chronic idiopathic
demyelinating polyneuropathy (AIDP/CIDP). We present a case of
pan-neurofascin antibody associated paranodal neuropathy with
serial electrodiagnostic findings.
A 61-year-old man presented with acute onset generalized weak-
ness and tiredness one day after the Covid-19 booster vaccination.
He had progressive sensory disturbance and weakness in his upper
limbs spreading to the lower limbs. He was treated with IVIg for
Guillain-Barré Syndrome (GBS) and discharged with some improve-
ment. Five days later, he developed rapidly progressive predomi-
nantly motor symptoms becoming quadriplegic and bedbound
within 9 days with no cranial or respiratory involvement. He was
found to have paranodal antibodies (neurofascin-155), consistent
with a pan-neurofascin antibody positive neuropathy. Although
the patient responded poorly to conventional immunomodulatory
therapies, he showed a rapid remarkable recovery with rituximab.
He was able to walk independently again within 3 weeks after
the second dose of rituximab. Electrodiagnostic studies showed
only subtle proximal demyelinating features with the initial pre-
sentation. The repeat study after his relapse showed a significant
deterioration with features of a generalized, non-length-dependent,
primarily demyelinating, sensorimotor polyneuropathy with con-
duction block, but the sural nerve biopsy showed axonal neuropa-
thy. Electrodiagnostic findings nearly normalized two months after
rituximab treatment with the resolution of the all the demyelinat-
ing features.
This case presentation highlights the importance of considering
nodal/paranodal neuropathy as a differential for AIDP/CIDP in an
early stage of the disease, especially in patients with atypical presen-
tation. In addition, it supports the currently available evidence that
more targeted treatment such as rituximab can have an excellent
outcome.
doi:10.1016/j.clinph.2022.07.447
TH-262. Risk and characteristics of Bell’s palsy as an adverse
event following COVID-19 vaccination
Sohyeon Kim *, Hung Youl Seok
Department of Neurology, Dongsan Medical Center, Keimyung University School of
Medicine, Daegu, Republic of Korea