Brain, Vol. 110, No. 1, 197-211, 1987
© 1987 Oxford University Press
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IDIOPATHIC CRANIAL POLYNEUROPATHY
A FIFTEEN-YEAR EXPERIENCE
From the Department of Neurology, Massachusetts General Hospital and Harvard Medical School Boston, MA, USA
Correspondence to:
Correspondence to: Dr Jorge L. Juncos, National Institute of Neurological and Communicative Disorders and Stroke, National Institutes of Health, 9000 RockvMe Pike, Bethesda, Maryland 20892, USA.
Fourteen cases of idiopathic multiple cranial neuropathy seen over fifteen years are reviewed and contrasted with 6 cases of the Tolosa-Hunt syndrome, the closest and better known clinical entity. The syndrome consists of subacute onset of facial pain preceding the onset of cranial nerve palsies. There were 5 men and 9 women aged 21 to 83 years. Eight of the 9 patients treated with corticosteroids showed improvement, with pain usually receding within 48 hours. The cerebrospinal fluid was abnormal in 7 of 12 cases, with either a mild pleocytosis or raised protein content. The cranial nerves most frequently involved were the third and sixth. Motor nerves were affected more than sensory. Corticosteroid therapy appeared to hasten recovery of function. Clinical features are shared by both syndromes, the resulting spectrum of illness probably reflecting diverse aetiologies. Historical perspectives, differential diagnosis and aetiological considerations are discussed so that a coherent prospective clinical approach to the problem can be developed.
Received December 12, 1985. Revised March 20, 1986. Accepted April 17, 1986.
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