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A case of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) with bilateral recurrent nerve palsy and primary alveolar hypoventilation--comparative studies of the histological findings of the two sural nerve biopsies with 9 years interval



A case of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) with bilateral recurrent nerve palsy and primary alveolar hypoventilation--comparative studies of the histological findings of the two sural nerve biopsies with 9 years interval



Rinsho Shinkeigaku 34(7): 712-716



A 27-year-old man noticed a tingling sensation in his fingers and toes in early 1982, at the age of 18 years, and his symptom gradually progressed in the following several months. In December 1982, based on neurological and laboratory examinations including the first sural nerve biopsy on the right side, a diagnosis of axonal sensory neuropathy of unknown etiology was made. During the following years, muscle weakness in the distal limbs and hoarseness developed and progressed, and the sensory impairments became gradually evident. On the second neurological examination in July 1991, he showed soft palate palsy and bilateral recurrent nerve palsy. The intrinsic muscles of hands and feet were atrophic, and pes cavus was noted bilaterally. There was mild to moderate weakness in the distal muscles of both limbs. Ankle jerk was absent and other tendon reflexes were decreased in both limbs. A mild to moderate decrease of both superficial and deep sensations with mild paresthesia was noted in both hands and feet. Routine laboratory findings were unremarkable. In blood gas analysis, hypercapnea and respiratory acidosis were found. Spirometry showed an increase of residual volume, and alveolar CO2-pulmonary ventilation response test suggested the presence of primary alveolar hypoventilation caused by hypofunction of the medullary respiratory center. In nerve conduction studies, motor nerve conduction velocities were moderately reduced in bilateral median and ulnar nerves. Distal latencies of M-waves were prolonged in bilateral median nerves. Temporal dispersion of M-wave was found in the left tibial nerve. The amplitudes of sensory action potentials were moderately reduced in bilateral median and sural nerves.

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Accession: 045035642

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PMID: 7955730


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