Motor neuron disease

Multiple sclerosis

Motor neuron disease

Motor neuron disease is degenerative disease which selectively affect motor tract fibers

(corticospinal tract+ anterior horn cell)

UMN signs LMN signs

Motor pathway

cortex motor area

Corticospinal fiber & corticobulber

AHC motor neuron disease

Peripheral nerves NMJ

muscle

pathology

Degeneration of the neurons

path physiology

Sporadic:90% unclear

Inherted:10% familial ALS,25% mutation in gene encoding copper zinc super oxide dismutase (SOD1)

course

Is progressive : median survival is approximately 3y

classification

Classic ALS (amyotrophic lateral sclerosis)..UMN+LMN signsothersProgressive muscular atrophyPrimary lateral sclerosisProgressive bulbar palsyProgressive pseudo bulbar palsy

Classic ALS

Mixed upper motor neuron + upper motor neuron signsEarly patient may exhibit only LMN signs or upper LMN signsWeakness begin a symmetrical and distally then spread to involve contiguous group of motor neuronsBulbar &pesudobulber palsy involvement ..dysphagea & dysarthria

Nooooooooooo

Cognitive

Sensory

Ocular

Autonomic Sphincter dysfunction

diagnosis

El Escorial criteria for dx

Definitive

Probable

possible

Electrophysiological

NCS: sensory..N

motor:normal or decreased amplitude

EMG: denervation

treatment

Riluzole :50 mg bid ( extend tracheotomy free survival by 2-3 months, not improving the survival or muscle strength

Supportive care physiotherapy, respiratory, swallowing…..

Multiple sclerosis

MS is the most disabling neurological condition of young adults

Epidemiology

Onset is typically in the mid 20s,although the dx may be delayed for several yearsThe ratio of f to m 1.77 to 1The incidence of MS in blacks residing in the united states is about 25% that of whitesHigh incidence includes all of Europe,North america,New Zealand,southern austeralia but the incidence also increasing in middle east

pathophysiogy

Inflamatory rxn causes variable tissue damage

Destruction of myelin producing cells (oligodendrocytes)

Some cells damaged without remyelination but oligodendrocytes precursors ..remyelinate..plaque

Risk factors

Genetic

Infection :viral

autoimmune

genetic

In general in the united states, the prevalence of MS is about ,1%

If a mother has MS,, her children's have a chance 3-5% .

If father has MS, his son has a1% chance & his daughter a 2% chance

Non identical twins has 3-4%

Identical twins:30%

Clinical presentation

Relapsing remitting: the commonest

(>one attack in >one site (multifocal)

Progressive relapsing

Primary progressive

Secondary progressive

diagnosis

Clinical :typical relapses come on over a few days, lasts for weeks or months ,and then clear, over 80% of patients begin with relapses

All central nervous system can be affected

Typical relapses

A-optic neuritis

B-myelopathy(spinal cord)

C-brain stem &cerebellar

Optic neuritis: clouding or blurring of central vision in one eye loss of measured activity, impair pupillary light reflex, some local pain made worse by eye movement…usually full recoveryMyelopathy: often sensory only; numbness &tingling from a certain level on the trunk on down through the rest of the body. if marked ..weaknessBrain stem

Each of these relapses may leave some residualAfter several attacks of various types, a patient may present common deficit Mild reduction in vision in one eyeNo conjugate eye movementsExtensor planter responses &inability to walk heel and toeReduced vibration sense in the legsUrgency of bladder function

Late stage deficit include: dementia, inability to stand or walk, slurred speech, ataxic, incontinence ,and marked sensory loss in hands &legs

Lehrmit sign

Athoufs phenomena

Diagnostic workup

MRI

Mri is now the dominant laboratory method of diagnosis in MSMS lesions are usually easily detected and often characteristic…Multiple bright lesion in T2 Contrast enhanced lesionShape :ovoidSize:>5mmSite: adjacent to the lateral ventricles, corpus callosum, cerebellum

LP: modest no of lymphocytes <50/mm,total protein <.8g/L,elevated immunoglobulin G(IgG), level oligoclonal banding on electrophoresis(80%)

Evoked potentials: VER,BAR,somatosensory evoked potential

diagnosis

McDonald criteria:

Confirm lesion >one site +> one attack

Diffrential diagnosis

Clinically:

Multiple infarctions

Autoimmune diseases

Vascuilities: behcets

Sarcidosis

Infection: chronic meningitis

Diseases that cause similar MRI pictures

Vascular: vascuilities,small vesseles disease,migraine

Infection:HIV.Lyme disease

Granulomtous :sarcidosis

ADEM

Treatment:

Definitive supportive

definitive

Six principles of management in multiple sclerosis1-relapses with significant impairment of function should be treated with high dose IV corticosteroid2-All relapsing remitting patients should be receiving long term immunomodulatory treatments3-Secondary progressive need aggressive tt early,late treatment <few years little benefit

4- primary progressive patients can not be expected to response to any treatment5-multiple sclerosis is a life long disease ,no specific time when to discontinue treatment once it started, if one modality of treatment fail or not tolerated ,another medication should be tried6-patients need to be watched for signs of disease activity by clinical or magnetic resonance monitoring or both.

Drug for acute phase

Methylpredinsolone 1g iv for 5d

Side effects:

Drug used for long term management

Interferon –B(avonex,betaseron,rebif.. decrease the risk of the attacks by 30%(sc.IM)

Side effects:

Depression, flu like, hepatitis

Copaxon: Widespread articaria

Supportive care symptomatic

Spasticity

Depression

Fatigue

Urinary urgency

pain