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An Unusual Subacute Progressive Motor Neuronopathy with Myasthenia-like Features

Published online by Cambridge University Press:  18 September 2015

J.H. Noseworthy ,
A.D. Rae-Grant  and
W.F. Brown
J.H. Noseworthy*
Affiliation:
Department of Clinical Neurological Sciences, University of Western Ontario, London
A.D. Rae-Grant
Affiliation:
Department of Clinical Neurological Sciences, University of Western Ontario, London
W.F. Brown
Affiliation:
Department of Clinical Neurological Sciences, University of Western Ontario, London
*
Department of Clinical Neurological Sciences, University Hospital, 339 Windermere Road, P.O. Box 5339, London, Ontario, Canada N6A 5A5
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Abstract:

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The initial presentation and clinical course of this 60-year old woman suggested a diagnosis of myasthenia gravis. The subsequent development of tongue fasciculations and the lack of response to treatment made a diagnosis of amyotrophic lateral sclerosis (ALS) more likely despite the presence of conjugate gaze paresis and the absence of many of the typical clinical and electromyographic (EMG) findings seen in this condition. The pathological findings were consistent with either a motor neuronopathy or an unusual variant of ALS. We review the clinical and pathological features of this unusual case in this report.

Type
Original Articles
Information
Canadian Journal of Neurological Sciences , Volume 15 , Issue 3 , August 1988 , pp. 304 - 309
Copyright
Copyright © Canadian Neurological Sciences Federation 1988

References

REFERENCES

1.Mossman, S, Vincent, A, Newsom-Davis, J. Myasthenia gravis with-out acetylcholine receptor antibody: A distinct clinical entity. Lancet 1986; 1: 116–19.CrossRefGoogle Scholar
2.Hirano, A. Progress in the pathology of motor neuron diseases. Prog Neuropathol 1973; 2: 181–215.Google Scholar
3.Brownell, B, Oppenheimer, DR, Trevor Hughes, J. The central ner-vous system in motor neuron disease. J Neurol Neurosurg Psychiat 1970; 33: 338–57.CrossRefGoogle Scholar
4.Carpenter, S. Proximal axonal enlargement in motor neuron disease. Neurology 1968; 18: 841–51.CrossRefGoogle ScholarPubMed
5.Spencer, PS, Schaumberg, HH: Classification of neurotoxic disease: A morphological approach, In: Spencer, PS, Schaumberg, HH, eds. Experimental and Clinical Neurotoxicology. Williams and Wilkins, Baltimore, 1980, p 92.Google Scholar
6.Bonduelle, M. Amyotrophic lateral stenosis. In: Vinken, PJ, Bruyn, GW, eds. Handbook of Clinical Neurology. Vol 22: System disorders and atrophies. New York: American Elsevier Publishing Co. 1975: 281–338.Google Scholar
7.Lambert, EH, Mulder, DW. Electromyographic studies in amy-otrophic lateral sclerosis. Proc Staff Meet Mayo Clin 1957; 32: 441–46.Google Scholar
8.Mulder, DW, Lambert, EH, Eaton, LM. Myasthenic syndrome in patients with amyotrophic lateral sclerosis. Neurology 1959; 9: 627–31.CrossRefGoogle ScholarPubMed
8.Simpson, JA. Disorders of neuromuscular transmission. Proc R Soc Med 1966; 59: 993–98.Google ScholarPubMed
10.Norris, F.H. Nerve stimulation studies in amyotrophic lateral sclero-sis. Fourth International Congress of Electromyography, Brussels, 1971; 106–7.Google Scholar
11.Denys, EH, Norris, FH Jr. Amyotrophic lateral sclerosis. Impairment of neuromuscular transmission. Arch Neurol 1979; 36: 202–5.CrossRefGoogle ScholarPubMed
12.Mulder, DW. Commentary. In: Mulder, DW, ed. The diagnosis and treatment of amyotrophic lateral sclerosis. Boston: Houghton Mifflin Professional Publishers 1980;79.Google Scholar
13.Van Bogaert, L. Contribution a la connaissance des troubles ocu-laires et vestibulaires dans la S.L.A. Rev Otoneuroophthalmol 1925; 3: 263–74.Google Scholar
14.Helfand, M. Progressive bulbar palsy: Its pathology and relation to amyotrophic lateral sclerosis. J Nerv Ment Dis 1933; 78: 362–80.CrossRefGoogle Scholar
15.Lawyer, T Jr, Netsky, MG. Amyotrophic lateral sclerosis (a clini-coanatomic study of fifty–three cases). Arch Neurol Psychiatry 1953; 69: 171–92.CrossRefGoogle Scholar
16.Jacobs, L, Bozian, D, Heffner, R, Barron, S. An eye movement disor-der in amyotrophic lateral sclerosis. Neurology 1981; 31: 1282–7.CrossRefGoogle Scholar
17.Leveille, A, Kiernan, J, Goodwin, JA, Antel, J. Eye movements in amyotrophic lateral sclerosis. Arch Neurol 1982; 39: 684–86CrossRefGoogle ScholarPubMed
18.Harvey, DG, Torack, RM, Rosenbaum, HE. Amyotrophic lateral sclerosis with ophthalmoplegia. A clinicopathological study. Arch Neurol 1979; 36: 615–17.CrossRefGoogle ScholarPubMed
19.Walsh, FB, Hoyt, WF: Heredofamilial and degenerative diseases. In: Walsh, FB, Hoyt, AB, eds. Clinical Neuro-Ophthalmology. Third Edition. Baltimore: Williams and Wilkins Co., 1969; 1: 936–38.Google Scholar
20.McGlone, J, Hudson, AJ. An eye movement disorder in ALS. Neurology 1983; 33: 254.CrossRefGoogle ScholarPubMed
21.Kushner, MJ, Parrish, M, Burke, A, et al. Nystagmus in motor neu-ron disease: Clinicopathological study of two cases. Ann Neurol 1984; 16: 71–77.CrossRefGoogle ScholarPubMed
22.Esteban, A, De Andres, C, Giménez-Roldan, S. Abnormalities of Bell’s phenomenon in amyotrophic lateral sclerosis. J Neurol Neurosurg Psychiatry 1978; 41: 690–98.CrossRefGoogle ScholarPubMed
23.Wall, M, Wray, SH. The one-and-a-half syndrome. A unilateral dis-order of the pontine tegmentum: A study of 20 cases and review of the literature. Neurology 1983; 33: 971–80.CrossRefGoogle Scholar