Name
AMYOTROPHIC LATERAL SCLEROSIS
DESCRIPTION
DETAIL
CAUSES & VARIETY : 1. ALS ( SPORADIC ) 2. ALS ( FAMILIAL ) INCIDENCE - 5 - 10 % -------------------------------------------------------------------------- D.D. : β’ Focal motor neuropathy β’ Cervical spondylosis β’ Lead intoxication β’ Spinal muscular atrophy (adult form) β’ Primary lateral sclerosis β’ Familial spastic paraparesis β’ Spinal multiple sclerosis β’ Tropical spastic paraparesis β’ Elevated levels of glutamate in CSF and serum β’ Anti-GM1 autoantibodies in low titer commonly found (of unclear significance) β’ Possibly reduced levels of nerve growth factor β’ Electromyography: denervation potentials (fibrillations, positive sharp waves) and often doublets are associated with prominent fasciculations (which suggest anterior horn cell dysfunction). Voluntary motor unit potentials have increased amplitude, long duration and/or polyphasic pattern. The recruitment pattern is reduced for the force generated and individual motor units have a high rate of discharge. β’ Muscle biopsy - will show groups of shrunken angulated muscle fibers (grouped atrophy) amid other groups of fi bers with a uniform fi ber type (fiber type grouping).
TYPENOTES
UNCOMMON BEFORE 40 YRS OF AGEAPPROPRIATE HEALTH CARE β’ Outpatient initially, may ultimately need nursing home placement and/or hospice β’ Supportive care for complicating emergencies (aspiration, respiratory failure). Use of a respirator is a major ethical dilemma. Consideration should be given to those with selective respiratory dysfunction DRUG(S) OF CHOICE Riluzole produces a slight prolongation in life expectancy by decreasing the release of glutamate POSSIBLE COMPLICATIONS β’ Aspiration pneumonia β’ Decubitus ulcers β’ Pulmonary embolism β’ Nutritional deficiency EXPECTED COURSE/PROGNOSIS β’ ALS usually terminates in death within five years β’ Patients predominantly manifesting progressive muscular atrophy have a better prognosis β’ There have been reports of spontaneous arrest of the disease
RELATED DISEASE
Not Available Disease
DISEASE
INVESTIGATION
MRI BRAIN ( FUNCTIONAL ), COMPLETE BLOOD COUNT, ELECTROMYOGRAPHY, MRI HEAD & SPINE, BIOPSY
[AMYOTROPHIC LATERAL SCLEROSIS]