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