[Late-onset adrenomyeloneuropathy perplexed with spondylosis. A case report].
We reported a 60-year-old man with late-onset adrenomyeloneuropathy (AMN). He had been well until 10 years before entry, when he noticed numbness in the legs with gait difficulty; symptoms worsened gradually with additional urinary disturbance. Transient improvement occurred after cervical and lumbar spinal operation under the diagnosis of spinal spondylosis, while his spastic gait got worse. Neurological examination on admission disclosed bilateral horizontal nystagmus, ataxic and spastic gait, increased patellar tendon reflexes, Chaddock sign, and impaired deep sense in the lower limbs with positive Romberg sign. Abnormal laboratory data included hypofunction of the adrenal cortex and elevated saturated very long chain fatty acids (VLCFAs). Serum cholestanol level was normal and anti-HTLV-1 antibody was negative. T2-weighted MRI showed a high signal intensity lesion in the occipital white matter along the optic radiation. Electrophysiological tests suggested a brainstem lesion on auditory brainstem response, thoracic or lumbar lesion on somatosensory evoked potential, and peripheral neuropathy on nerve conduction study. In the present case, it should be emphasized that the determination of serum VLCFAs unveiled the diagnosis of AMN in old patients with spinal spondylosis or without apparent clinical symptoms of adrenocortical insufficiency.
Sakai
H
H
Department of Neurology, Kanazawa University School of Medicine.
Hayashi
S
S
Nitta
E
E
Takamori
M
M
D000326:Adrenoleukodystrophy / Q000150:complications* / Q000175:diagnosis; D017668:Age of Onset; D003937:Diagnosis, Differential; D005231:Fatty Acids, Unsaturated / Q000097:blood; D006801:Humans; D008297:Male; D008875:Middle Aged; D013128:Spinal Osteophytosis / Q000150:complications*
D005231:Fatty Acids, Unsaturated