amyotrophic lateral sclerosis ( als ) is a devastating neurodegenerative disease which affects both motor function and extramotor systems . according to the revised el escorial criteria for the diagnosis of als , the presence of signs for the affection of both upper motor neurons ( umns ) in the primary motor cortex and lower motor neurons ( lmns ) in brainstem and spinal cord is mandatory , and the disease must be progressive . because of varied clinical phenotypes of als , it is difficult to differentiate it from other als - mimicking conditions . electromyography can be used for the detection of lmn involvement in addition to the clinical examination . on the other hand , umn signs must be visible at the clinical examination while electrophysiological transcranial motor stimulation abnormalities are not included in making the diagnosis of als according to the el escorial criteria . magnetic resonance imaging ( mri ) is the radiological investigation of choice with the recent advances of diffusion tensor imaging , presenting a promising technique for early detection of alterations in the motor cortex and pyramidal tracts . herein , we report a case of a 9-year - old male who presented to the department of neurology with complaints of progressive increase in weakness of bilateral lower limb , poor grip in bilateral upper limb , and difficulty in walking since last 2 years . there was no history of any muscle pain , trauma , fever , recent vaccination , dog bite , respiratory tract or gastrointestinal tract infection . physical examination demonstrated decreased power in the bilateral lower limb ( 3/5 in the right lower limb and 2/5 in the left lower limb ) and upper limb ( 4/5 in the right hand and 4/5 in the left hand ) , no signs of dermal rashes , or hypertrophy of muscles . mri of the brain was performed using 1.5 tesla scanner ( ge signa excite , usa ) . t2-weighted imaging and additional fluid attenuating inversion recovery sequence showed hyperintense signals in the posterior limb of internal capsule [ figure 1a and b ] involving corticospinal tract ( cst ) which was found to extending up to midbrain . ( a ) t1-weighted imaging , black arrow shows hyperintensity in the posterior limb of internal capsule ( b ) t2-weighted imaging , black arrow shows hyperintensity in the posterior limb of bilateral internal capsule ( c ) t2-weighted imaging , black arrow shows hyperintensity in the posterior bilateral internal capsule and midbrain ( corticospinal tract ) with classical wine glass appearance areas of abnormal signal intensity on t1- and t2-weighted images in the cst have been previously reported in patients with als . other diseases such as ischemic processes , demyelinating diseases , and infections may involve the pyramidal tracts . however , bilateral symmetrical involvement associated with the characteristic clinical findings clinches the diagnosis of als . the degeneration of motor neurons may result in a cellular loss and axonal edema as previously shown on electronic microscopy . so far , imaging methods had been of limited use because of their unsatisfactory results . approximately , 90% of als are sporadic with 10% having familial predisposition , but majority of the cases of juvenile als ( jals ) are familial . most of the jals are autosomal recessive , though autosomal dominant inheritance patterns have been described . recessive forms of jals have been mapped to chromosome regions 2q33 and 15q12 - 21 . no effective treatment for the disease have been found till date , though thyrotropin - releasing hormone , ganglioside therapy , and plasmapheresis have been tried in adults without much benefit . jals is a diagnosis that should be suspected in a child presenting with a combination of umn and lmn signs . mri facilitates reaching the correct diagnosis by showing the typical involvement of the cst and the wine glass appearance . mri plays as an important diagnostic tool for reaching up to a diagnosis and for prognosticating the disease .