Intramedullary Astrocytoma Presenting as Spinal Muscular Atrophy

Abstract

We present a 6-year-old patient with a spinal cord tumor who had been followed with the diagnosis of spinal muscular atrophy since the age of 23 months. Reasons for reevaluating the diagnosis of spinal muscular atrophy were the early onset of scoliosis, the slight asymmetry in weakness of extremities, and the appearance of urinary retention in the last 3 days. Magnetic resonance imaging revealed a very long, intramedullary tumor extending from the level of the seventh cervical segment to the conus medullaris, later reported to be a grade I astrocytoma. We therefore recommend that magnetic resonance imaging, a noninvasive and sensitive technique for intraspinal pathologies, be performed in every patient with an atypical form of spinal muscular atrophy. (J Child Neurol 1993;8:354-356).

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References

Wessel HB : Spinal muscular atrophy. Pediatr Ann 1989;18: 421-427.

Goutlières F. , Bogicevic D. , Aicardi J. : A predominantly cervical form of spinal muscular atrophy. J Neurol Neurosurg Psychiatry 1991 ;54:223-225.

Yamada M. , Kano M. , Chida K. , et al: Asymptomatic benign familial spinal muscular atrophy with hypertrophy of the calves and high creatine kinase levels. J Neurol Neurosurg Psychiatry 1988;3:452-453.

Armstrong RM , Fogelson MH , Silberberg DH : Familial proximal spinal muscular atrophy . Arch Neurol 1966;14:208-212.

Munsat TL , Skerry L. , Korf B. , et al: Phenotypic heterogeneity of spinal muscular atrophy mapping to chromosome 5q11.2-13.3 (SMA 5q). Neurology 1990;40:1831-1836.

De Visser M. , De Visser BWO, Verbeeten B Jr: Electromyographic and computed tomographic findings in five patients with monomelic spinal muscular atrophy. Eur Neurol 1988;28:135-138.

Karni A. , Navon R. , Sadeh M. : Hexosaminidase A deficiency manifesting as spinal muscular atrophy of late onset. Ann Neurol 1988;24:451-453.

Wood S. , Shukin RJ , McGillivray BC , et al: A grandpaternally derived de novo deletion within Xp21 initially presenting in carrier females diagnosed as Kugelberg-Welander syndrome. Am J Med Genet 1988;29:419-423.

Hausmanowa-Petrucewicz I. : Electrophysiological findings in childhood spinal muscular atrophies. Rev Neurol (Paris) 1988;144:716-720.

10.

Merlini L. , Granata C. , Bonfiglioli S. , et al: Scoliosis in spinal muscular atrophy: Natural history and management . Dev Med Child Neurol 1989;31:501-508.

11.

Pearn JH , Wilson J. : Chronic generalised spinal muscular atrophy of infancy and childhood . Arch Dis Child 1973;48:768-774.

12.

Fredericks RK , Elster A. , Walker FO : Gadolinium-enhanced MRI: A superior technique for the diagnosis of intraspinal metastases. Neurology 1989;39:734-736.

13.

Kramer ED , Rafto S. , Packer RJ , Zimmerman RA : Comparison of myelography with CT follow-up versus gadolinium MRI for subarachnoid metastatic disease in children. Neurology 1991;41:46-50.

14.

Epstein FJ , Wisoff JH : Intramedullary tumors of the spinal cord, in McLaurin RL , Schut L , Venes JL , Epstein F (eds): Pediatric Neurosurgery, 2nd ed. Philadelphia, WB Saunders, 1989, pp 428-442.

15.

DeSousa AL , Kalsbeck JE , Mealey J. Jr , et al: Intraspinal tumors in children. J Neurosurg 1979;51:437-445.