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Diagnosis of AB variant GM2 gangliosidosis.

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Spinal Canal Hemorrhage
and CT Scanning
raphy or C T scanning with contrast medium should be the
preferred study in such situations.
Mark P. Carol, MD, Bruce S. Harris, MD,
and M. A. Whelan, PhD, M D
Reference
Computed tomographic (CT) scanning has become increasingly used in the diagnosis of spinal cord disorders, and unenhanced scans have been shown to detect the presence of
epidural blood in the spinal canal 11’1.W e report our experience in a patient with hemorrhagic spinal cord compression.
A 20-year-old man was admitted to the hospital with a
serious systemic illness that subsequently proved to be due
to disseminated herpes simplex. He had an associated
hepatitis and coagulopathy. Because of persistent high fever
and mental status changes, lumbar puncture was performed
after the administration of platelets and fresh frozen plasma.
Cerebral spinal fluid was obtained without difficulty: protein
and glucose levels were normal and there were 230 red
blood cells/cm3 and no white blood cells. Ten hours later, the
patient suffered the acute onset of paraplegia, with a sensory
level in the low thoracic region. C T scanning was performed
without contrast agent. Despite the presence of some motion
artifact, the scan was considered technically adequate to rule
out the presence of a surgical lesion causing spinal cord displacement. The possibility of subarachnoid blood was raised,
however. One week later, repeat lumbar puncture was undertaken but spinal fluid could not be obtained, suggesting
the presence of a spinal block. Repeat C T scan, again done
without contrast agent, after window manipulation revealed a
questionable increase in density in the subarachnoid space at
T11 and T12. Marked cord compression was not suggested.
Two days later, cisternal puncture yielded hazy, reddish
brown fluid with xanthochromia and greater than 18,000 red
blood cellsicmi. Metrizamide was instilled into the thecal sac
and a myelogram performed. The myelogram was interpreted as indicating epidural compression from T4 through
T10, with scalloping of the spinal cord outline and displacement of the spinal cord and dura mater. No contrast material
passed below T10. C T scanning showed obvious displacement of the spinal cord by a low-density mass, with no contrast agent seen below T12. Because of the prolonged time
course, surgical decompression was not undertaken.
We believe our experience suggests that C T scanning
without instillation of contrast agent may be inadequately
sensitive to the presence of subarachnoid, subdural, and/or
epidural blood in the spinal canal, even in the acute phase
when it should be relatively hyperdense, and that myelog-
102 Annals of Neurology Vol 19 No 1 January 1986
Cooperstown, NY
1. Beatty RM, Winston KR: Spontaneous cervical hematoma: a consideration of etiology. J Neurosurg 61: 143-148, 1984
Diagnosis of AB Variant,
GM2 Gangliosidosis
Suresh Kotagal, M D
In the article by Kivlin and associates {2) leukocyte lysosomal enzyme assay is suggested as the diagnostic test for
the AB variant of GM2 gangliosidosis. The hallmark of this
disorder, however, is GM2 ganglioside accumulation in the
face of quantitatively and qualitatively normal leukocyte hexosaminidase A and B owing to deficiency of the activator
protein for hexosaminidase A. This is indicated in the paper
by de Baecque and associates 111, quoted by the authors.
Histological evidence of ganglioside storage in conjunctiva
or brain is therefore also required before diagnosis of the
AB variant can be established.
Division of Pediatric Neurology
St. Louis Uniwwsity Medical Center
1221 South Grand Blwd
St. Louis, MO 63104
Reply
Jane D. Kivlin, M D
In Table 2 of the article by myself and Drs Sanborn and
Myers [2], the items “conjunctival or brain biopsy” should
be added under the necessary diagnostic tests with the item
“AB variant.”
Department of Ophthalmology
University of Utah Medical Center
50 No. Medical Dr
Salt Lake City, U T 84132
References
1. deBaecque CM, Suzuki K, Rapid I, et al: GM2gangliosidosis, AB
variant. Acta Neuropathol (Bed) 33:207-226, 1975
2. Kivlin JD, Sanborn GE, Myers GG: The cherry-red spot in TaySachs and other storage diseases. Ann Neurol 17:356-360, 1985
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