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January 1968

Early Childhood Cerebral Lipidosis With Prominent Myoclonus: Ultrastructural and Histochemical Studies of a Cerebral Biopsy

Author Affiliations

Little Rock, Ark
From the departments of pathology (Dr. Richardson) and pediatrics (Dr. Bornhofen), University of Arkansas Medical Center, Little Rock.

Arch Neurol. 1968;18(1):34-43. doi:10.1001/archneur.1968.00470310048004

DISTINCTIVE membranous cytoplasmic lipid inclusions have been demonstrated in ultrastructural studies of patients with infantile amaurotic idiocy (Tay-Sachs disease).1-3 This suggests that other lipidoses may also have characteristic morphological lipid configurations which would aid in their differentiation.

The classification of cerebral lipidoses in older children is confused.4 These have generally been divided into late infantile amaurotic idiocy (Bielschowsky-Jansky disease) and juvenile amaurotic idiocy (Batten-Spielmeyer-Vogt disease). Whether these lipidoses are separate entities or whether they are manifestations at different ages of a similar disease is disputed.

Attempts have been made to correlate the ultrastructure of the lipid with the clinical syndromes termed late infantile amaurotic idiocy,5,6 systemic late infantile lipidosis,7 juvenile amaurotic idiocy,8,9 and juvenile lipidosis.10 Due to varying terminology, similar lipid inclusions are often described under different clinical names. No consistent pattern on electron microscopic examination has emerged.

This report presents histochemical and

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