[A case of acute polyradiculoneuritis with multiple cranial nerve palsy and cerebral lesion--possible evidence of encephalo-myelo-radiculo-neuropathy].
We report a case of acute polyradiculoneuritis with multiple cranial nerve palsy and cerebral lesions. A boy, born on July 26, 1987, developed unusual sensation on the extremities, backache and sleep disturbance on June 23, 1996. On July 2, following a complaint of blindness he developed a convulsion and was admitted to our hospital. Neurological examination revealed intact consciousness, severe external ophthalmoplegia, bifacial palsy and generalized areflexia. On the next day, flaccid tetraplegia and respiratory dysfunction developed and progressed without disturbance of consciousness. After tracheal intubation he was under mechanical ventilation. A lumbar puncture examination showed clear CSF with increased protein 166 mg/dl. no cells and normal myelin basic protein. Serum antibodies against gangliosides (GM1, asialo-GM1, GD1b and GQ1b) were not detected. A posterior tibial nerve conduction velocity was mildly delayed with disappearance of F- wave. On the other hand, very slow background activity was shown by EEG, extensive focal hypoperfusion of cerebral blood flow by SPECT and supratentorial multiple high intensity lesions by T2 weighted MRI of the brain. There were no abnormal signals in the brainstem and cerebellum on MRI. His condition dramatically improved after plasmapheresis. The abnormal findings of SPECT and MRI promptly disappeared within 3 weeks, although abnormal signs on EEG persisted. He was successfully weaned off the respirator and recovered strength of the limbs. He was discharged on August 28, 1996, with supported walk and bifacial palsy, then he completely recovered by 7 months. The condition of case was compatible with 'encephalo-myelo-radiculo-neuropathy', a disease entity that had previously been reported in a few patients in whom with Guillain-Barré or Fisher syndrome and cerebral symptoms co-existed.
Department of Pediatrics, Miyazaki Medical College.
N
N
Itokazu
Y
Y
Kodama
S
S
Kontani
S
S
Inoue
T
T
Sugimoto
T
T
Ohi
D000208:Acute Disease; D002648:Child; D003389:Cranial Nerve Diseases / Q000150:complications*; D004673:Encephalomyelitis, Acute Disseminated / Q000150:complications* / Q000628:therapy; D006801:Humans; D008297:Male; D010243:Paralysis / Q000150:complications*; D010951:Plasma Exchange; D011129:Polyradiculoneuropathy / Q000150:complications* / Q000628:therapy