Background:Hirayama disease (HD) is benign focal amyotrophy of the distal upper limbs, often misdiagnosed as motor neuron disease. Routine magnetic resonance imaging (MRI) is often reported normal.Objective:To study the clinicoradiological profile of hand wasting in young males.Materials and Methods:Patients presenting with insidious-onset hand wasting from March 2008 to May 2011 were evaluated electrophysiologically. Cervical MRI in neutral position was done in 11 patients and flexion contrast imaging was done in 10 patients.Results:All patients were males less than 25 years of age, with median age 23 years, except one patient who was 50 years old. Duration of illness was 3 months to 3 years. All (100%) had oblique amyotrophy, four (36%) cold paresis, 10 (91%) minipolymyoclonus and three (27%) had fasciculations. Regional reflexes were variably absent. Two patients (18%) had brisk reflexes of lower limbs with flexor plantars. Electromyography (EMG) showed chronic denervation in the C7-T1 myotomes. Neutral position MRI showed loss of cervical lordosis in 10/11 (91%), localized lower cervical cord atrophy in 9/11 (82%), asymmetric cord flattening in 11/11 (100%) and intramedullary hyperintensity in 2/11 (18%); flexion study showed loss of dural attachment, anterior displacement of dorsal dura, epidural flow voids in 9/10 (90%) and enhancing epidural crescent in 10/10 (100%). Clinical profile, imaging and electrophysiological findings of the patient aged 50 years will be described in detail as presentation at this age is exceptional. Collar therapy slowed progression in most cases.Conclusion:Clinical features of HD corroborated well with electrophysiological diagnosis of anterior horn cell disease of lower cervical cord. While dynamic contrast MRI is characteristic, routine studies have a high predictive value for diagnosis. Prompt diagnosis is important to institute early collar therapy.
Since its original description by Keizo Hirayama in 1959, “juvenile muscular atrophy of the unilateral upper extremity” has been described under many nomenclatures from the east. Hirayama disease (HD), also interchangeably referred to as monomelic amyotrophy, has been more frequently recognised in the west only in the last two decades. HD presents in adolescence and young adulthood with insidious onset unilateral or bilateral asymmetric atrophy of hand and forearm with sparing of brachioradialis giving the characteristic appearance of oblique amyotrophy. Symmetrically bilateral disease has also been recognized. Believed to be a cervical flexion myelopathy, HD differs from motor neuron diseases because of its nonprogressive course and pathologic findings of chronic microcirculatory changes in the lower cervical cord. Electromyography shows features of acute and/or chronic denervation in C7, C8, and T1 myotomes in clinically affected limb and sometimes also in clinically unaffected contralateral limb. Dynamic forward displacement of dura in flexion causes asymmetric flattening of lower cervical cord. While dynamic contrast magnetic resonance imaging is diagnostic, routine study has high predictive value. There is a need to lump all the nomenclatures under the rubric of HD as prognosis in this condition is benign and prompt diagnosis is important to institute early collar therapy.
We hereby report an extremely unusual case of cystic angiomatous meningioma in the CPA region in a 58-year-old male patient. He presented with complaints of headache, repeated episodes of vomiting and increasing unsteadiness of gait. Neuroimaging showed a large multicystic left-sided tentorial tumor projecting into the cerebellum and CPA with contrast-enhancing peripheral solid rim. He underwent a left retromastoid craniectomy and total excision of the tumor. Histopathology revealed an angiomatous meningioma with predominant microvascular component and extensive cystic changes. Immunopositivity for epithelial membrane antigen (EMA), vimentin and S-100 protein proved vital in excluding a hemangioblastoma.
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