[Infantile onset chronic inflammatory demyelinating polyneuropathy with clinical course of 23 years; a case report].
We report a 23-year-old female with infantile onset chronic inflammatory demyelinating polyneuropathy. Muscle weakness was noticed when she was 1 year old, and, together with sensory disturbances, gradually progressed in an asymmetric manner. Nerve conduction studies disclosed slowing of conduction velocities, temporal dispersion, and decreased amplitude of compound muscle action potentials, the degrees of which were markedly different amongst different nerves even in the same limbs. The sural nerve biopsy showed various degrees of nerve fiber amongst different fascicles. Marked inter-nerve and intra-nerve differences of involvement and clinical improvement after steroid therapy supported the diagnosis of chronic inflammatory demyelinating polyneuropathy. It should be noted that even after a long clinical course of 23 years, her clinical symptoms remained asymmetrical and multi-focal lesions could be clearly demonstrated.
Sakai
K
K
Department of Neurology, Tokyo Metropolitan Neurological Hospital.
Kato
S
S
Komori
T
T
Tanabe
H
H
D000328:Adult; D017668:Age of Onset; D000893:Anti-Inflammatory Agents / Q000008:administration & dosage; D002908:Chronic Disease; D003711:Demyelinating Diseases / Q000175:diagnosis* / Q000188:drug therapy / Q000503:physiopathology; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D008775:Methylprednisolone / Q000008:administration & dosage; D009431:Neural Conduction; D010525:Peripheral Nerves / Q000503:physiopathology; D011115:Polyneuropathies / Q000175:diagnosis* / Q000188:drug therapy / Q000503:physiopathology; D011239:Prednisolone / Q000008:administration & dosage; D013497:Sural Nerve / Q000473:pathology
D000893:Anti-Inflammatory Agents; D011239:Prednisolone; D008775:Methylprednisolone