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Correlation of Clinical and Biochemical Data

Yoshiyuki Suzuki, MD; Allen C. Crocker, MD; Kunihiko Suzuki, MD

Arch Neurol. 1971;24(1):58-64.

	Since this article does not have an abstract, we have provided the first 150 words of the full text PDF and any section headings.

IN 1964, Landing et al¹ established "familial neurovisceral lipidosis" as a clinicopathological entity. The same disease has also been called generalized gangliosidosis,^2-7 late infantile systemic lipidosis,^8-11 G_M1-gangliosidosis,^3,12-18 familial infantile amaurotic idiocy with visceral involvement,^19,20 biochemically special form of infantile amaurotic idiocy,^21,22 and Landing disease.^5,14,17,23 The disease is characterized by generalized accumulation of a monosialoganglioside, G_M1 (ganglioside nomenclature of Svennerholm²⁴ ), and visceral accumulation of keraton sulfate-like mucopolysaccharide.^12,18 Enzymatically, deficiency of β-galactosidase has been demonstrated in various organs.^{4,5,7,14,16-18,23,25,26}

From the clinical viewpoint, there appear to be two subtypes.¹⁰ Patients in the infantile age category often exhibit abnormal facies, full forehead, and radiologically detectable bony changes reminiscent of Hurler's syndrome. On the other hand, some older patients in the "late infantile" age group show few or no clinical or radiologie signs of Hurler's syndrome.

The present study was . . . [Full Text PDF of this Article]

Author Affiliations
Philadelphia; Boston; Philadelphia

From the Department of Neurology, University of Pennsylvania School of Medicine, Philadelphia (Drs. Y. Suzuki and K. Suzuki); the Mental Retardation Unit, Children's Hospital Medical Center and the Department of Pediatrics, Harvard Medical School (Dr. Crocker), Boston.

Footnotes
Accepted for publication Sept 12, 1970.

Reprint requests to Department of Neurology, Johnson Pavilion, University of Pennsylvania School of Medicine, Philadelphia 19104 (Dr. Y. Suzuki).

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