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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 dec amp
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…..
Diagram weakness approach
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 tt <few years little benefit
4- primary progressive patients can not be expected to response to any tt5-multiple sclerosis is a life long disease ,no specific time when to discontinue tt once it started,if one modality of tt fail or not tolerated ,another medication shouled be tried6-patients need to be watched for signs of disease activity by clinical or magnetic resonance monitring or bothor both.
Drug used for long term management
Interferon –B(avonex,betaseron,rebif..dec the risk of the attacks by 30%(sc.IM)
Side effects:
Depression,flu like,hepatitis
Copaxon:
Widespread articaria
Other immunsuppretion
Drug for acute phase
Methylpredinsolone 1g iv for 5d
Side effects:
Supportive care symptomatic)
Spasticity
Depression
Fatigue
Urinary urgency
pain