- See:
EMG in Demyelinating Neuropathies:
- Discussion:
- is a relapsing, and commonly progressive disorder of the white matter of the CNS;
- is the most common demyelinating disease to affect the spinal cord and brain;
- 80% of cases involve the cord, and 20-33% show symptoms referable to cord alone;
- lesions may involve any region of cord, regardless of anatomy, although the cervical cord is involved more often;
- often begins in early adulthood and has a variable prognosis:
- 10 % of patients may do well for more than 20 years;
- about 70 percent will have secondary progression;
- one half will have difficulty walking after 15 yrs of dz;
- initial clinical findings may include proprioceptive sensory loss and upper motor neuron signs;
- other symptoms may include ataxia, tremor, or nystagmus;
- MRI:
- multifocal lesions of various ages
- involvment of the periventricular white matter, brain stem, cerebellum, and spinal cord white matter;
- presence of gadolinium-enhancing lesions indicates acute sites of inflammatory demyelination;
-
relapsing-remitting multiple sclerosis
- this type present in 80 percent of MS patients
- typically begins in the second or third decade of life
- female predominance of approximately 2:1.
- symptoms often progress over several days, the stabilize and may improve;
- clinical findings:
- sensory disturbances;
- unilateral optic neuritis;
- diplopia (internuclear ophthalmoplegia);
- Lhermitte's sign
- limb weakness;
- clumsiness
- gait ataxia
- neurogenic bladder and bowel symptoms;
- onset of symptoms post partum
- Uhthoff's symptom: symptomatic worsening with increases in body temperature
- trigeminal neuralgia;
- episodic clumsiness;
- dysarthria;
- tonic limb posturing;
- aphasia
- recurrent seizures
- visual-field loss
- early dementia
- extrapyramidal phenomena (chorea and rigidity)
- cognitive impairment
- depression
- emotional lability
- dysarthria
- dysphagia
- vertigo
- progressive quadriparesis
- ataxic tremors
- pain
- sexual dysfunction
- spasticity
- treatment:
- corticosteroids
- steroids may improve recovery but the effectiveness may diminish over time;
-
primary progressive MS:
- 20 % of affected patients have primary progressive MS;
- patients have a gradually progressive course;
- similar incidence among men and women;
- present with a slowly evolving upper-motor-neuron syndrome of the legs
- worsens gradually with clinical findings:
- quadriparesis
- cognitive decline
- visual loss
- brain-stem syndromes
- sexual disfunction
- bladder and bowel dysfunction;
Medical Progress: Multiple Sclerosis
John H. Noseworthy, Claudia Lucchinetti, Moses Rodriguez, Brian G. Weinshenker
The New England Journal of Medicine -- September 28, 2000 -- Vol. 343, No. 13
