PubMed1999
The patient, a 65-year-old woman, had liver cirrhosis, and had blood transfusion at the age of 49 and 56. Early in September, 1989, she gradually developed numbness of the legs, staggering gait, and apathy with hallucination. In October, she became incontinent and unable to stand, and was admitted to Konan Hospital. On admission, she was disoriented with poor comprehension. Cranial nerves were intact except for horizontal nystagmus on lateral gaze. She had generalized areflexia without pathological reflex. Muscular forces were fairly preserved. Superficial sensations were diminished in the upper limbs as well as below Th-7 level. Deep sensation was abolished in the distal parts of the extremities with athetotic finger postures on arm rising. She had urinary and fecal incontinence. Results of routine laboratory examinations were non-contributory. Chest CT scan and sputum cytology were normal. CSF contained one cell/microliter, 95 mg/dl of protein with positive oligoclonal IgG bands. Anti-HTLV-I antibody was positive in serum and CSF. Urodynamic studies showed neurogenic bladder of supranuclear type. MNCV was slightly decreased. SNAP and SEP were not evoked. On sural nerve biopsy, the density of myelinated fibers was 720/mm2, and that of unmyelinated fibers, 26,978/mm2. ABR and VEP were abnormal. EEG showed diffuse theta waves with paroxysmal delta and sharp waves. T2-weighted MR images of the brain showed patchy areas of high signal intensity in the cerebral white matter. Soon after administration of methylprednisolone, her consciousness became clear. EEG normalized in 4 months. Twenty months after the onset, she became ambulant with crutch, but still has dysuria and sensory deficits in the hands and lower limbs. The possible relationship between encephalomyeloneuritis and HTLV-I infection was discussed.