MS/ALS

• MS CLIN


  Weakness, numbness, tingling, or unsteadiness in a limb. Charcot's neuro triad (dysarthria, nystagmus, intention tremor), Lhermitte's sign, poor concentration/critical thinking/anxiety
• Other MS symptoms


  • Constipation, incontinence, sexual dysf., troubles with talking, eating, swallowing, optic neuritis, double visition, weake/spasms/tremors/ataxia/paralysis, numb, pins/needles, paresthesias
• Relapsing-remitting MS


  Bouts of autoimmune attacks mo-yrs apart, residual permanent damage. Disabilities do NOT increase between episodes
• Secondary progressive MS


  Starts as RRMS but attacks become constant and disabilities progress
• Primary progressive MS


  One instant attack with superimposed bouts so there is a faster progression of disability
• MS PE


  • Episodic neuro sx, younger than 55yo, single pathologic lesion cannot explain clinical findings, multiple foci is best seen w MRI
• MS imaging


  • MRI→Hypointense T1, hyperintense T2 lesions, ≥ one lesion in periventricular, juxtacortical, infratentorial, spinal cord, Gadolinium-enhanced, non enhanced lesion simultaneously, Dawson's Fingers
• Understand the most appropriate imaging for the brain and spinal cord for MS diagnosis and surveillance/relapses
• MS PATHO


  Autoimmune demyelinating disease of nerve cells in brain/spinal cord that shows w neuro disorders. Type 4 hypersensitivity involving T cells, b cells and macrophages… these cells breach the BBB → upregulation of adhesion molecules and inflammatory cytokines → Produce myelin-specific antibodies triggering inflammatory demyelination→Results in loss of oligodendrocytes→Hallmark plaque is characterized by loss of myelin sheets, relative preservation of axons, and glial (astrocytic) scar formation) →Loss of myelin disrupts nerve conduction → sx
• MS RISK


  Genetic origination but can be brought on by environmental factors
• Age group most affected by MS

  Females <55yo (20-40yo)
• MS Treatments


  • RRMS=Corticosteroids, cyclophosphamide, IV immunoglobulin, Plasmapheresis: removing antibodies, Immunosuppressants
  • Progressive MS: Manage symptoms (e.g., urinary incontinence), PT, cognitive rehabilitation therapy, vitamin D
  • Ocrelizumab slows disability progression in PPMS
• ALS CLIN


  Weakness and variable wasting of affected muscles w/o sensory changes. Happens between 30-60yo. Degeneration of the anterior horn cells in the spinal cord, the motor nuclei of the lower CNs and the corticospinal/corticobulbar pathways, cognitive decline (in a pattern consistent with frontotemporal dementia), a pseudobulbar affect, or parkinsonism
• ALS PATHO


  Altered RNA processing (SOD1 mutation) → degeneration/gliosis of axons in anterior/lateral columns of spinal cord… Genetic one gene 9 (autosomal dominant pattern)
• MUST HAVE UMN (hyperreflexia/spasticity), LMN (muscle atrophy/fasciculation)
• ALS progression


  Variable patterns of onset, LMN degeneration with proximal limbs → paralysis and death. Limb onset, bulbar onset, progressive muscular atrophy, primary lateral sclerosis
• ALS DX


  Evidence of LMN, UMN degeneration and progressive s/sx in a region or to other regions… no electro/patho causes or neuroimaging answer
• ALS TX= none :(