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Brain, Vol. 115, No. 2, 495-520, 1992
© 1992 Oxford University Press

research-article

PRIMARY LATERAL SCLEROSIS: CLINICAL FEATURES, NEUROPATHOLOGY AND DIAGNOSTIC CRITERIA

C. E. PRINGLE1, A. J. HUDSON1, D. G. MUNOZ2, J. A. KIERNAN3, W. F. BROWN1 and G. C. EBERS1

1Department of Clinical Neurological Sciences Canada 2Department of Pathology (Neuropathology) Canada 3Department of Anatomy, University of Western Ontario Canada

Correspondence to: Correspondence to Dr G. C Ebers, Department of Clinical Neurological Sciences, University Hospital, 339 Windermere Road, London, Ontario, Canada N6A 5A5.

Eight patients with a homogeneous syndrome of progressive symmetric spinobulbar spasticity were studied. Clinical features were limited to those associated with dysfunction of the descending motor tracts and included spastic quadriparesis, pseudobulbar affect, spastic dysarthria, hyper-reflexia and bilateral Babinski signs. Lower motor neuron findings were absent and higher cognitive function preserved. Median age of onset was 50.5 yrs and median disease duration was 19 yrs Neuropathologic features (including morphometric analysis) in the single autopsied case confirmed the selective involvement of the motor cortex. There was complete absence of Betz cells from layer 5 of the precentral cortex and the remaining pyramidal cells were significantly smaller than those seen in normal controls. Magnetic resonance imaging (MRI) revealed atrophy of the precentral gyrus and positron emission tomography (PET) scans showed diminished glucose [18F]flurodeoxyglucose uptake in the pericentral cortex. Magnetic motor cortex stimulation revealed markedly prolonged central motor conduction times. The literature is reviewed and diagnostic criteria for primary lateral sclerosis based on clinical, laboratory and imaging features are proposed

Received May 16, 1991. Revised October 6, 1991. Accepted January 26, 1992.


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