Tutorial Neuro Part II...

by KT

OVERVIEW
Myasthenia gravis Parkinsonism Epilepsy Multiple sclerosis Normal pressure hydrocephalus Motor neuron disease Muscle disease GBS/CIDP Polyneuropathy, CMT AED+status epilepticus

Myasthenia gravis

Investigation
Tensilon test Ice test Single ber electromyography Repetitive nerve stimulation Ab testing: AChR Ab, Anti-MuSK

RNS

AChR antibodies 80% in generalized MG 55% in Ocular MG Anti-MuSK: orofacial+respiratory, rarely ocular

Drug exacerbating
Curare Penicillamine Botox uoroquinolone, macrolide, aminoglycoside Magnesium

Treatment
anticholinesterase medications thymectomy: thymoma, if no-->generalized, onset<50 immunosuppression plasma exchange IVIG .. S/E: aseptic meningitis, allergic

Lambert eaton syndrome

Presynaptic Antibodies to P/Q type voltage-gated Ca channel Fatigable weakness Autonomic sx: xerostomia, blurred vision Association Treatment: 3,4 Diaminopyridine

Exam
25 yo 1 wk 3 PE: bilateral facial palsy, feet and wrist drop, absent DTR treatment?
high dose methylprednisolone high dose broad spectrum ATB plasmapheresis interferon immunosuppressant

CIDP
slowly progressive motor>sensory, hyporeexia 8 weeks Associated: HIV, SLE, MGUS, hepatitis, IBD, lymphoma Tx: IVIg 2g/kg 5 days + Prednisolone

Parkinsonism

Parkinsonism
Parkinsons disease: loss of dopaminergic neurons of the substan4a nigra Secondary cause: drugs, vascular, NPH Parkinson plus: MSA, PSP, CBD, DLB Neurodegenerative: Wilsons dz, Huntington dz

Hemiballism
large amplitude movement, one side of body proximal muscle Subthalamic nucleus Cause: Stroke, toxoplasma Tx: haloperidol, propanolol, clonazepam

Seizure

Side effects

Side effects

Status epilepticus

Contraception
Drugs decrease efcacy carbamazepine phenytoin phenobarbital topiramate

TIA: ABCD2

Multiple sclerosis

Clinical manifestations

Ancillary symptoms
Heat sensitivity (Uhthoffs symptom) Lhermitte Paroxysmal Trigeminal neuralgia, hemifacial spasm

Investigation

MRI CSF Visual evoked potential

MRI

Treatment

acute: methyprednisolone disease modifying tx: b interferon

Neuromyelitis optica

Devic syndrome How does it differ from MS?

Motor neuron disease

fasciculations, LMN/ UMN Pseudobulbar palsy: dysarthria, dysphagia, emotional liability- laughing/crying Tx: Mostly symptomatic ALS-- Riluzole

Motor neuron disease

UMN Primary lateral sclerosis Hereditary spastic Spinal muscular atrophy paraparesis Kennedy syndrome Adrenomyeloneuro pathy LMN UMN+LMN

ALS

Adrenomyeloneuropathy
over accumulation of very long chain fatty acid (VLCFA) brain, adrenal gland, spinal cord

Kennedy disease
X-linked spinobulbar muscular atrophy mid-adult life androgen insensitivity: gynecomastia, reduced fertility expanded CAG repetition

Myopathy

Progressive muscular dystrophies