ATAXIA
GAD AB
PHENOTYPE:
ATAXIA/ PARKINSONISM/ PSP
STIFF PERSON SYNDROME
STARTLE MYOCLONUS
OPSOCLONUS-MYOCLONUS
ORTHOSTATIC TREMOR
LIMBIC ENCEPHALITIS/ MTLE
ABNORMAL SACCADES
VELOCITY/LATENCY/ACCURACY
SACCADE INITIATION/ EARLY UPGAZE PALSY
NYSTAGMUS
DOWN/ UP BEAT NYSTAGMUS
PERIODIC ALTERNATING NYSTAGMUS
FLUTTER/OPSOCLONUS/ STIFF EYES
>50YRS: 8%+VE GAD POSITIVE
NMDA AB
MUTISM/ AUTONOMIC DYSFUNCTION
CENTRAL HYPOVENTILATION
STEREOTYPE (PRESENT IN SLEEP)
FACE DYSKINESIA
LIMB AND TRUNK DYSKINESIA
PSYCHOSIS PRESENTATION
FEVER/ HEADACHE/ VOMITING
POST HSV/ VZV INFECTION
NEOPLASIA:
OVARY/ BREAST
SMALL CELL LUNG CARCINOMA
CASPR2 AB
EPISODIC ATAXIA
NEUROMYOTONIA/ MYOKYMIA
LOWER LIMB ACTION MYOCLONUS
SEGMENTAL/ PROPRIOSPINAL MYOCLONUS
SMALL CALL CARCINOMA LUNG
THYMOMA
CRMP5
PARKINSONISM/ ATAXIA
AUTONOMIC DYSFUNCTION
MSP-P PHENOTYPE
BILATERAL OPTIC NEUROPATHY
SMALL CELL CARCINOMA LUNG
MRI: BASAL GANGLIA HYPERINTENSITY
PERIVENTRICULAR/ INTERNAL CAPSULE HYPERINTENSITY
AMPHIPHYSIN
NEUROPATHY/ ENCEPHALOPATHY
MYELOPATHY/STIFF PERSON SYNDROME
CEREBELLAR ATAXIA
NEOPLASIA: SMALL CELL LUNG CARCINOMA
GFAP AB
MENINGO-ENCEPHALO-MYELITIS
ATAXIA (30%)
TREMORS(30%)
PSYCHIATRIC(30%)
AUTONOMIC (30%)
DPPX6 AB
COGNITION
THORACIC STIFFNESS
PARKINSONISM (PSP PHENOTYPE)
BRAIN STEM INVOLVEMENT
GI DYSAUTONOMIA
DIARRHOEA/ WEIGHT LOSS
TREMOR/ MYOCLONUS/ HYPEREKPLEXIA
SEIZURES/ PARKINSONISM/ ATAXIA
IgLON5 AB
SLEEP PROBLEMS:
OSA/RBD/NREM/ STRIDOR
OCULAR:
SACCADIC INTRUSIONS/ VSGP
SYMPTOMS:
COGNITION/SEIZURES/ENCEPHALOPATHY
PARKINSONISM/DYSTONIA/ATAXIA
OROMANDIBULAR DYSTONIA
OROFACIAL DYSKINESIA
HYPEREKPLEXIA
MYORHYTHMIA/ TREMOR
AUTONOMIC DYSFUNCTION
DIABETES INSIPIDUS/ MYOKYMIA
PHENOTYPES
CBS/ PSP/MSA/HD/SPS/MND
SYMPTOMS
BULBAR 80%
SLEEP 80%
COGNITION 50%
ATAXIA 70%
CHOREA 30%
BRADYKINESIA/ RIGIDITY 30%
DYSTONIA 30%
TREMOR 20%
MYOCLONUS/ MYORHYTHMIA/MYOKYMIA 30%
RX: IMMUNOTHERAPY EFFECTIVE IN 13%
OPSOMYOCLONUS ATAXIA
NEUROBLASTOMA
CHILDREN
LAB: URINE VMA/ METANEPHRINES INCREASED
RI AB
AGE OF ONSET >40YRS
SUBACUTE ONSET PARKINSONISM
PSP PHENOTYPE
OCULOPALATAL-MYOCLONUS
NEOPLASIA: BREAST/ OVARY
GAD AB
PHENOTYPE
ATAXIA/ PARKINSONISM/ PSP
STIFF PERSON SYNDROME
STARTLE MYOCLONUS
HEAD RETRACTION JERK
OPSOCLONUS-MYOCLONUS
ORTHOSTATIC TREMOR
LIMBIC ENCEPHALITIS/ MTLE
ABNORMAL SACCADES
VELOCITY/LATENCY/ACCURACY
SACCADE INITIATION/ EARLY UPGAZE PALSY
NYSTAGMUS
DOWN/ UP BEAT NYSTAGMUS
PERIODIC ALTERNATING NYSTAGMUS
FLUTTER/OPSOCLONUS/ STIFF EYES
>50YRS: 8% GAD POSITIVE
NMDA AB
MUTISM/ AUTONOMIC DYSFUNCTION
CENTRAL HYPOVENTILATION
STEREOTYPE (PRESENT IN SLEEP)
FACE DYSKINESIA
LIMB/TRUNK DYSKINESIA
PSYCHOSIS PRESENTATION
FEVER/ HEADACHE/ VOMITING
POST HSV/ VZV INFECTION
NEOPLASIA: OVARY/ BREAST
SMALL CELL LUNG CARCINOMA
GLYCINE RECEPTOR AB
HYPEREKPLEXIA/ PERM
GABA-B RECEPTOR AB
SEIZURES/ MYORHYTHMIA
KELCH LIKE (KLH11 AB)
RHOMBENCEPHALITIS
ATAXIA/DEAFNESS/VERTIGO
TESTICULAR SEMINOMA
BRAINSTEM/ MTL / DENTATE HYPERINTENSITY
POST-INFECTIOUS
IDIOPATHIC
RI AB
AGE OF ONSET >40YRS
SUBACUTE ONSET PARKINSONISM
JAW CLOSING DYSTONIA
PSP PHENOTYPE
OCULOPALATAL-MYOCLONUS
NEOPLASIA: BREAST/ OVARY
YO AB
BREAST/ OVARY CARCINOMA
RAPIDLY PROGRESSIVE
POOR RESPONSE TO IMMUNOTHERAPY
HU AB
SMALL CELL LUNG CARCINOMA
TR/mGLuR1 AB
HODGKIN LYMPHOMA
VGCC AB
LAMBERT EATON SYNDROME
ZIC4 AB
SMALL CELL LUNG CARCINOMA
PCA2 (MAP-1B IgG )
SMALL CELL LUNG CARCINOMA (80%)
ATAXIA/ COGNITION/ NEUROPATHY
TPO AB
STROKE/ ENCEPHALOPATHY
CSF ABNORMAL 80%
PROTEIN INCREASED
GQ1b AB
CANOMAD
CHRONIC ATAXIA NEUROPATHY OPHTHAMOPLEGIA, IGM PARAPROTEINAEMIA
COLD AGGLUTININS
MILLER FISCHER SYNDROME
ATAXIA/OPHTHALMOPARESIS
BICKERSTAFF ENCEPHALITIS
SPASTICITY/ UP GOING PLANTERS
GALOP SYNDROME
GAIT ATAXIA LATE ONSET POLYNEUROPATHY
LAB: M-BAND IGM
ATAXIA NEUROPATHY
PCA2 (MAP-1B IgG )
ATAXIA/ COGNITION/ NEUROPATHY
SCLC 80%
NF-L IgG
ATAXIA/ COGNITION/ NEUROPATHY
MYELOPATHY/ AUTONOMIC
AP3B2-IgG
ATAXIA/ COGNITION/ NEUROPATHY
MYELOPATHY/ AUTONOMIC
ROMBENCEPHALITIS
MA2
CATAPLEXY/ VSGP
HU AB
SMALL CELL LUNG CARCINOMA
CRMP5/ CV2 AB
PARKINONISM/ ATAXIA/ CHOREA
AUTONOMIC DYSFUNCTION
MSP-P PHENOTYPE
BILATERAL OPTIC NEUROPATHY
SMALL CELL CARCINOMA LUNG
MRI: BASAL GANGLIA HYPERINTENSITY
PERIVENTRICULAR/ INTERNAL CAPSULE HYPERINTENSITY
ARHGAP AB
IgLON5 AB
SLEEP PROBLEMS:
OSA/RBD/NREM/ STRIDOR
OCULAR:
SACCADIC INTRUSIONS/ VSGP
SYMPTOMS:
COGNITION/SEIZURES/ENCEPHALOPATHY
PARKINSONISM/DYSTONIA/ATAXIA
OROMANDIBULAR DYSTONIA
OROFACIAL DYSKINESIA
HYPEREKPLEXIA
MYORHYTHMIA/ TREMOR
AUTONOMIC DYSFUNCTION
DIABETES INSIPIDUS/ MYOKYMIA
PHENOTYPES
CBS/ PSP/MSA/HD/SPS/MND
SYMPTOMS
BULBAR 80%
SLEEP 80%
COGNITION 50%
ATAXIA 70%
CHOREA 30%
BRADYKINESIA/ RIGIDITY 30%
DYSTONIA 30%
TREMOR 20%
MYOCLONUS/ MYORHYTHMIA/MYOKYMIA 30%
RX: IMMUNOTHERAPY EFFECTIVE IN 13%
DPPX6 AB
COGNITION
THORACIC STIFFNESS
PARKINSONISM (PSP PHENOTYPE)
BRAIN STEM INVOLVEMENT
GI DYSAUTONOMIA
DIARRHOEA/ WEIGHT LOSS
TREMOR/ MYOCLONUS/ HYPEREKPLEXIA
SEIZURES/ PARKINSONISM/ ATAXIA
NEUROCHONDRIN IgG
MCP AND PONS HYPERINTENSITY
SEPTIN-5 AB
OSCILLOPSIA/ VERTIGO
KELCH LIKE PROTEIN (KLH11 AB) AB
RHOMBENCEPHALITIS
DEAFNESS/VERTIGO
TESTICULAR SEMINOMA
BRAINSTEM/ MTL / DENTATE HYPERINTENSITY
PERM
GAD/ GLYCINE-RECEPTOR/ DPPX6 AB
LIMB OR AXIAL PAINFUL SPASMS
RIGIDITY/ NYSTAGUS/ OPSOCLONUS
CORTICAL MYOCLONUS/ STARTLE
DEAFNESS/ OPHTHALMOPARESIS
DYSARTHRIA
APNEA/ HYPERSOMNOLENCE
ASSOCIATED WITH THYMOMA
LAB: CSF PLEOCYTOSIS
GQ1b AB
CANOMAD
CHRONIC ATAXIA NEUROPATHY OPHTHAMOPLEGIA, IGM PARAPROTEINAEMIA
COLD AGGLUTININS
MILLER FISCHER SYNDROME
ATAXIA/OPHTHALMOPARESIS
BICKERSTAFF ENCEPHALITIS
OTHERS
LISTERIA
CLIPPERS
PARANEOPLASTIC
PARAINFECECTIOUS
CNS LYMPHOMA
SARCOIDOSIS
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CATAPLEXY
DROP ATTACKS
MA2
CATAPLEXY/ VSGP
CASPR2 AB
EPISODIC ATAXIA
NEUROMYOTONIA/ MYOKYMIA
LGI1 AB
FACIOBRACHIAL DYSTONIC SEIZURES
DROP/ ATONIC ATTACKS
HYPONATREMIA
EPISODIC BRADYCARDIA