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Brain Advance Access published online on September 29, 2006
Brain, doi:10.1093/brain/awl258
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© The Author (2006). Published by Oxford University Press on behalf of the Guarantors of Brain. All rights reserved. For Permissions, please email: journals.permissions@oxfordjournals.org
Received April 25, 2006
Revised April 18, 2006
Accepted April 18, 2006

Article

Facial onset sensory and motor neuronopathy (FOSMN syndrome): a novel syndrome in neurology

S. Vucic 1, D. Tian 2, P. Siao Tick Chong 3, M. E. Cudkowicz 3, E. T. Hedley-Whyte 2, and D. Cros 3 *

1 Prince of Wales Medical Research Institute, Randwick, Australia
2 C. S. Kubik Laboratory for Neuropathology, Department of Pathology Massachusetts General Hospital, Harvard Medical School, Boston, MA, USA
3 Department of Neurology, Harvard Medical School, Boston, MA, USA

* To whom correspondence should be addressed.
D. Cros, E-mail: dcros{at}partners.org


  Abstract

A ‘syringomyelia-like’ syndrome has been infrequently reported in neurological disorders such as Tangiers disease and lepromatous leprosy. This study reports a novel ‘syringomyelia-like’ syndrome in four adult male patients, which we have termed facial onset sensory and motor neuronopathy, or FOSMN syndrome, that appears to have a neurodegenerative aetiology. Clinical, neurophysiological and pathological data of four patients were reviewed, including the autopsy in one patient. Four male patients (mean age at onset 43), initially developed paraesthesiae and numbness in a trigeminal nerve distribution, which slowly progressed to involve the scalp, neck, upper trunk and upper limbs in sequential order. Motor manifestations, including cramps, fasciculations, dysphagia, dysarthria, muscle weakness and atrophy developed later in the course of the illness. Neurophysiological findings revealed a generalized sensory motor neuronopathy of caudally decreasing severity in all four patients. Autopsy in one patient disclosed loss of motoneurons in the hypoglossal nucleus and cervical anterior horns, along with loss of sensory neurons in the main trigeminal sensory nucleus and dorsal root ganglia. FOSMN syndrome appears to be a slowly progressive neurodegenerative disorder, whose pathogenesis remains to be determined.

Keywords: FOSMN syndrome; syringomyelia; motoneuron disease; sensory neuropathy.
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