[Striatal involvement on MRI in adrenomyeloneuropathy].
Adrenomyeloneuropathy (AMN), a clinical variant of child adrenoleukodystrophy (ALD), is an adult-onset progressive disorder which presents spastic paraparesis with peripheral nerve involvement and affects mainly the pyramidal tracts from the brainstem to the spinal cord. We report a case of AMN in which serial MRI showed unusual development of areas of high signal in the right striatum. The patient was in good health until the age of 12, when he began to lose his hair. At age 25 he started to have progressive gait disturbance and erectile impotence. In his first admission to our hospital at age 33, he showed diffuse baldness. He was intelligent but childish. His cranial nerves were normal. Muscle strength was weak (3-4/5) in the lower extremities. Deep tendon reflexes were hyperactive in the lower extremities while normal in the upper extremities. Babinski signs were elicited bilaterally. Pinprick and vibratory sensation was impaired in the lower legs. Proprioceptive sensations were normal. Co-ordination was intact. There were urinary incontinence and impairment of erection with preserved libido and ejaculation. Routine laboratory data including hematological studies, serum chemistry and urinalysis were all normal except for mild hyperlipidemia. Serum cortisol response to ACTH was low and serum levels of very long chain fatty acids were increased. Nerve conduction studies were abnormal and consistent with peripheral polyneuropathy. A biopsy specimen of left sural nerve revealed a mild loss of myelinated fibers with thinning of the myelin. These findings and the clinical features confirmed the diagnosis of AMN. MRI in SE2000/40 scans at age 34 disclosed areas of high signal in the bilateral internal capsules.(ABSTRACT TRUNCATED AT 250 WORDS)
Fukutake
T
T
Department of Neurology, School of Medicine, Chiba University, Japan.
Sakakibara
R
R
Katayama
K
K
Nakajima
M
M
Hirayama
K
K
D000326:Adrenoleukodystrophy / Q000175:diagnosis* / Q000503:physiopathology; D000328:Adult; D003342:Corpus Striatum / Q000473:pathology*; D002549:Diffuse Cerebral Sclerosis of Schilder / Q000175:diagnosis* / Q000503:physiopathology; D016057:Evoked Potentials, Auditory, Brain Stem; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D009128:Muscle Spasticity; D010243:Paralysis / Q000209:etiology; D010523:Peripheral Nervous System Diseases / Q000503:physiopathology