[A case of agammaglobulinemia with chronic enteroviral meningomyelitis].

We report a 30-year-old man with agammaglobulinemia and chronic aseptic meningomyelitis. The patient was diagnosed as having X-linked recessive agammaglobulinemia at 4 years of age and gammaglobulin supplementation was started. He had TIA-like episodes several times since 25 years of age. He developed difficulty in micturition and impotence at 29 years of age. Neurological examination revealed bilateral deafness, contracture of knee joints, slight weakness and areflexia in the lower extremities, Babinski sign and dysuria. There was sensory disturbance in the lower extremity on the left. There was not consciousness disturbance or meningeal irritation sign. The cerebrospinal fluid findings included pleocytosis and increase in protein. Enterovirus RNA was detected in the cerebrospinal fluid by the modified polymerase chain reaction (PCR) method. MRI of lower spinal cord showed syrinx formation in the lumbosacral cord and CT of the brain showed bilateral temporal lobe atrophy and temporoparietal subdural fluid collection on the left. 123I-IMP SPECT showed decrease in the cerebral blood flow in the whole brain. EEG showed diffuse slow activity, suggesting the subclinical encephalopathy. Chronic enteroviral meningoencephalitis with agammaglobulinemia (CEMA) is one of the complications of agammaglobulinemia. However, myelitis without apparent encephalopathy is very rare. To our knowledge, there have been no reports of spinal sylinx formation in CEMA.