Amyotrophic lateral sclerosis: MRI
Case Report: 35 yr old lady referred for altered behavior , suggests a possible "Wineglass " appearance of symmetrical T2 hyper intensity along the internal capsules, brainstem , with rest of the corticospinal tracts grossly unremarkable at present. Further investigation including clinical, EMG & Lab data correlation is being planned .
Teaching points by Dr MGK Murthy
1. This greek word is completely self explanatory
A (Negative) Myo (Muscle) Trophic (Nourishment) Lateral (spinal cord lateral parts involved) Sclerosis (Terminal degree of degeneration = Scarring )
2. Other names include , Lou Gehring ( Named after ,famous Basket ball player victim) and Charcot disease.(responsible for initial description of Anterior horn cells of spinal cord ,being etiology for the disease)
3. A variety of Motor neuron disease( poor prognosis ,average survival of 3-5 years) of Upper Motor neuron (giant pyramidal variety (BETZ) cells of motor cortex) and Lower Motor neuron ( including anterior horn cells of spinal cord) degeneration(basic pathology is Wallerian degeneration)
4. 40-60 yrs usual age with 2 different patterns
(a) familial (genetic mutations)
(b) Sporadic (unknown, and may be genetic+ environmental).
MRI shows symmetrical T2 hyper intensity of corticospinal tracts (from precentral region, corona radiata, Internal capsule, crus cerebri , pons), with typical Wine glass shape (better appreciated on Coronal)
5. Juvenile variety unusual , and shows cellular and axonal edema. Electromyography is helpful and Long TE , variety of MRS shows decreased NAA and NA/Cr ratio. Treatment is primarily supportive
Amyotrophic lateral sclerosis: MRI Reviewed by Sumer Sethi on Tuesday, April 04, 2017 Rating: