NMOSD & MOGAD
2. Recovery
NMOSD: generally relapsing >90%, uni/bil optic neuritis, infrequency antecedant infection, Risk of poor recovery
MOGAD : 50% monophasia and 50% relapsing, often bilateral involvement, common antecedent infection, generally good but residual sphincter and erectile problems
Name 4 orobuccolignual chorea
•Tardive dyskinesia (dopamine receptor-blocking medications)
•Neuroacanthocytosis syndromes
•Neuroferritinopathy
•Parneoplastic syndromes (NMDA)
•Lesch-Nyhan syndrome
•Acquired hepatocerebral degeneration
•Anosmia
•Constipation
•REM sleep behaviour disorder
•Depression
Clinical phenotype of CIDP
5 Variants
symmetric proximal and distal upper and lower extremity weakness and sensory loss affecting at least two limbs with areflexia that is progressive over a time period greater than 2 months.
Focal, Motor, Sensory, Multifocal, and distal
5 predisposing conditions associated with CVST
Alcohol consumption ◆ Hypercholesterolemia ◆ Hyperhomocysteinemia ◆ Antiphospholipid antibodies present and antiphospholipid syndrome ◆ Autoimmune disease ◆ Anemia ◆ Malignancy (particularly within the first year of cancer diagnosis as well as among patients with hematologic malignancies)19 ◆ Pregnancy and the puerperium (a 2019 case-control study found a nearly 11-fold adjusted increased risk of cerebral venous thrombosis [CVT] with higher risk during the first 6 weeks postpartum as compared to 7 to 14 weeks)20 ◆ Factor V G1691A polymorphism ◆ Methylenetetrahydrofolate reductase (MTHFR) C677T polymorphism ◆ Prothrombin G20210A polymorphism ◆ Protein C deficiency ◆ Protein S deficiency ◆ Antithrombin III deficiency ◆ Obesity (in women who used oral contraceptives, being overweight or obese was associated with an increased risk of CVT in a dose-dependent manner) ◆ Elevated factor VIII serum levels ◆ Behçet disease2
MRI features of MOGAD and NMOSD
1.Optic neuritis
2. Transverse myelitis
NMOSD: chiasmal
LETM, elongated or patchy enhancement
MOGAD- long lesiona >50%, perineural enhancement
LETM, often with short lesions, conus involvement and H sign axially, acute enhancement in 50% cases
4 casuses of hemichorea
•Contralateral subthalamic nucleus
•Nonketotic hyperglycemia
•Sydenham chorea
•Polycythemia vera
60 year old male with diagnosis of PD on Sinemet 100/25 TID and rotigotine patch. He started gambling and spent $50000 on shopping.
1. What is this called?
2. What is the next step?
1. Impulse control disorder
2. Wean off the dopamine agonist
Name 5 differential diagnoses/mimics for CIDP
anti-MAG neuropathy
antiparanodal and antinodal chronic immune-mediated demyelinating neuropathy
MMN
CANOMAD (chronic ataxic neuropathy, ophthalmoplegia, immunoglobulin IgM paraprotein, cold agglutinins, and disialosyl antibodies)
POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal protein, and skin changes) syndrome,
hereditary demyelinating neuropathies,
Clinical features of Brown Sequard Syndrome
Ipsilateral UMN pattern of weakness below the level of the lesion
Ipsilateral lower motor neuron (LMN) pattern of weakness at the level
Ipsilateral vibration and position sensation loss below the level
Contralateral pain and temperature sensation loss below the level (one to two segments below the level of the lesion)
May have radicular pain (from root irritation) or hemianesthesia (dorsal root compromise) at the level of the lesion
May have ipsilateral loss of sweat below the lesion and ipsilateral Horner syndrome (if cervical) due to autonomic involvement
6 core Clinical characteristics of NMOSD
1. Acute or subacute optic neuritis
2. Acute or subacute myelitis
3. Area postrema syndrome, defined as an otherwise unexplained episode of hiccups, nausea,or vomiting lasting for≥48 hours and that do not recede with symptomatic medications
4. Acute or subacute brainstem syndrome
5. Symptomatic narcolepsy or acute diencephalic clinical syndrome with diencephalic MRI lesions typical of AQP4-NMOSD around the third ventricle
6. Symptomatic cerebral syndrome with brain MRI lesions typical of AQP4-NMOSD(extensive white matter lesions around the lateral ventricles)
1. Chorea- acanthocytosis is commonly associated with - important clinical sign on examination
2. Treatable chorea -
3. Most common genetic cause of chorea in children
1. Feeding dystonia- lingual protrusion dystonic
rubber man syndrome
2. Wilson
3. Benign hereditary chorea
Three motor phenotypes of PD
CIDP albuminocytologic dissociation,
normal nucleated cell count
elevated protein levels.
What is ICANS
How does it present
Immune effector cell–associated neurotoxicity syndrome
biphasic, with the first peak within 5 days of chimeric antigen receptor (CAR) T-cell administration, often occurring with cytokine release syndrome, and a second delayed peak occurring 3 to 4 weeks later
6 core clinical characteristics of MOGAD
1. Acute or subacute optic neuritis
2. Acute or subacute myelitis
3. ADEM, defined as acute or subacute polyfocal neurologic deficits with encephalopathy, accompanied by multifocal T2 lesions on MRI.
4. Cerebral monofocal or polyfocal deficits without encephalopathy
5. Acute or subacute brainstem or cerebellar deficits
6. Cerebral cortical encephalitis with seizures, defined as MRI evidence of FLAIR cortical hyperintensity, often with enhancement of the overlying meninges, accompanied by cerebral irritability (eg, encephalopathy, headache, focal neurologic deficit) in addition to seizures.
Seen in ?
Inappropriate generalized atrophy excessive for age.
The frontal horns are enlarged with a box-shaped appearance.
The frontal horn to intercaudal distance ratio is significantly reduced.
1. When do you consider genetic testing in PD
2. Adequate levodopa challenge test?
1. <50 years patient with family history of PD
2. consider using a total daily levodopa equivalent dose equal to or greater than 600 mg/d and an adequate per-dose strategy to unmask potential improvement.
Name 3 paranodal and nodal antibodies
Most effective treatment
nodal neurofascin 186 (NF186)/ paranodal proteins contactin-1 (CNTN1), contactin-associated protein 1 (CASPR1), or neurofascin 155 (NF155).
reflect an immunoglobulin G4 (IgG4)–mediated response and respond only partially or not at all to typical first-line treatments for CIDP.
more likely to be adults, to be male, and to present with a severe and symmetric, distal, motor-predominant rapidly progressive disease.
Tremor and sensory ataxia more notably manifest in this patient population as compared with patients with typical CIDP.
Nephrotic syndrome has been reported to occur more commonly in patients with CNTN1antibodies.
Panneurofascin antibody positivity has been associated with underlying lymphoma, myeloma, and leukemia.
Rituximab
The two most common complications of CNS presentation associated with immune checkpoint inhibitors
The two most common complications of PNS presentation
Meningitis and Encephalitis
Myopahty and Myasthenia Gravis
Eculizumab
Satralizumab
inebulizumab
C5 inhibitor
IL-6 inhibitor- tocilizumab
CD 19 antibody
Huntington disease
1. Caused by
2. Complete penetrance ?
3. Juvenile onset?
autosomal dominant disorder caused by a triplicate CAG expansion in the short arm of chromosome. geneHTT
2. 40 -60 or more
3. 60 or more, parkinsonism and seizure
1.clear and dramatic response to dopaminergic therapy
2.levodopa-induced dyskinesias
3. resting tremor of a limb
4. olfactory loss (anosmia or hyposmia)
5. cardiac sympathetic denervation on metaiodobenzylguanidine (MIBG) scintigraphy.
Which antibodies need to be checked?
asymmetric, distal upper limb predominant with pure motor symptoms.
Often the finger flexors are relatively spared, and foot drop may be the first symptom in one-third of patients.
The diagnosis of MMNrequires motor nerve involvement in at least two nerves for more than 1 month.
Anti-GM1 antibodies are seen in some but not all patients with MMN. In patients with MMN
Clinically, no sensory symptoms are present.
Reflexes are often absent or diminished in the affected limb.
Electrophysiology often demonstrates demyelinating features with conduction block in the motor nerves
Diagnostic criteria for IIH
Modified Dandy Criteria for the Diagnosis of Idiopathic Intracranial Hypertension
Signs and symptoms of increased intracranial pressure (eg, headaches, transient obscurations of vision, papilledema, pulse synchronous tinnitus, visual loss)
No altered level of consciousness nor localizing neurologic signs, except unilateral or bilateral sixth nerve palsy
Increased intracranial pressure with normal CSF composition
Neuroimaging without any structural lesion or venous sinus thrombosis
No other etiology explaining intracranial hypertension