A case of neurofibromatosis 2 presenting with a mononeuritis multiplex

• Published in: Journal of neurology, neurosurgery and psychiatry Vol. 55; no. 5; pp. 391 - 393
• Main Authors: Kilpatrick, T J, Hjorth, R J, Gonzales, M F
• Format: Journal Article Conference Proceeding
• Language: English
• Published: London BMJ Publishing Group Ltd 01.05.1992; BMJ; BMJ Publishing Group LTD
• Subjects: Adult; Biological and medical sciences; Biopsy; Hereditary Sensory and Motor Neuropathy - diagnosis; Hereditary Sensory and Motor Neuropathy - genetics; Hereditary Sensory and Motor Neuropathy - pathology; Humans; Magnetic Resonance Imaging; Male; Medical sciences; Muscles - innervation; Nerve Fibers - pathology; Neuritis - diagnosis; Neuritis - genetics; Neuritis - pathology; Neurofibromatosis 2 - diagnosis; Neurofibromatosis 2 - genetics; Neurofibromatosis 2 - pathology; Neurologic Examination; Neurology; S100 Proteins - analysis; Sural Nerve - pathology; Tomography, X-Ray Computed; Tumors of the nervous system. Phacomatoses; Case study; Human; Nervous system diseases; Tumor; Nevritis; Inaugural sign; Neurofibromatosis Recklinghausen
• ISSN: 0022-3050; 1468-330X
• DOI: 10.1136/jnnp.55.5.391

A patient with neurofibromatosis 2 had an asymmetrical peripheral neuropathy. A nerve biopsy specimen revealed neurofibromatous changes, and the neuropathy may have been a direct consequence of neurofibromatosis. An apparent clinical response to immunosuppressive treatment and plasma exchange is also reported.