title: A Case of Apoplexy Attack-Like Neuropathy due to Hereditary Neuropathy with Liability to Pressure Palsies in a Patient Diagnosed with Chronic Cerebral Infarction.
abstract: Hereditary neuropathy with liability to pressure palsies is an inherited disease associated with the loss of a copy of the PMP22 gene. The condition leads to mononeuropathy due to compression and easy strangulation during daily life activities, resulting in sudden muscle weakness and sensory disturbance, and displaying symptoms similar to cerebrovascular diseases. We report the case of an 80-year-old man with left paralysis due to chronic cerebral infarction. His medical history indicated remarkable recovery from about 4 months after the onset of left hemiplegia with predominant involvement of the fingers. Despite subsequent recurrent monoplegia of the upper or lower limbs, brain magnetic resonance imaging consistently revealed only previous cerebral infarction in the right corona radiata without new lesions. Medical examination showed reduced deep tendon reflexes in his extremities on both the healthy and hemiplegic sides. Nerve conduction studies showed delayed conduction at the bilateral carpal and cubital tunnels and near the right caput fibulae. Genetic analysis revealed loss of a copy of the PMP22 gene. Thus, he was diagnosed with a cerebral infarction complicated by hereditary neuropathy with liability to pressure palsies. Stroke patients develop sudden muscle weakness and sensory disturbance. However, if such patients have no hyperactive deep tendon reflexes and show atypical recovery of paralysis that does not correspond to findings of imaging modalities, nerve conduction studies and genetic analysis may be necessary, considering the complication of hereditary neuropathy with liability to pressure palsies.
authors.affiliation: Department of Rehabilitation Medicine, University of Occupational and Environmental Health, Kitakyushu, Japan. Electronic address: akih815@med.uoeh-u.ac.jp.
authors.forename: Akiko
authors.initials: A
authors.lastname: Hachisuka
authors.affiliation: Department of Rehabilitation Medicine, University of Occupational and Environmental Health, Kitakyushu, Japan.
authors.forename: Yasuyuki
authors.initials: Y
authors.lastname: Matsushima
authors.affiliation: Moji Medical Center, Kitakyushu, Japan.
authors.forename: Kenji
authors.initials: K
authors.lastname: Hachisuka
authors.affiliation: Department of Rehabilitation Medicine, University of Occupational and Environmental Health, Kitakyushu, Japan.
authors.forename: Satoru
authors.initials: S
authors.lastname: Saeki
mesh_terms: D000369:Aged, 80 and over; D001176:Arthrogryposis / Q000150:complications* / Q000175:diagnosis / Q000235:genetics / Q000503:physiopathology; D002544:Cerebral Infarction / Q000150:complications* / Q000175:diagnosis / Q000503:physiopathology; D002908:Chronic Disease; D004576:Electromyography; D020233:Gait Disorders, Neurologic / Q000150:complications / Q000175:diagnosis / Q000503:physiopathology; D020022:Genetic Predisposition to Disease; D015417:Hereditary Sensory and Motor Neuropathy / Q000150:complications* / Q000175:diagnosis / Q000235:genetics / Q000503:physiopathology; D006801:Humans; D017404:In Situ Hybridization, Fluorescence; D008279:Magnetic Resonance Imaging; D008297:Male; D009043:Motor Activity; D018908:Muscle Weakness; D009185:Myelin Proteins / Q000235:genetics; D009460:Neurologic Examination; D010641:Phenotype; D012684:Sensory Thresholds
chemical_list: D009185:Myelin Proteins; C073687:PMP22 protein, human
keywords: HNPP; PMP22 gene; TIA; cerebral infarction