Pathology

Lectin histochemical studies were performed on selected formalin-fixed, paraffin-embedded tissues of patients affected with the O variant of GM2-gangliosidosis (i.e., Sandhoff's disease). The purpose was to identify specific sugar residues of undegraded "stored" substances in cytoplasm of affected cells. We studied neural tissues from 13 patients, visceral tissues from four patients, and placentae from three affected fetuses. Neurons in all 13 cases studied stained with Concanavalia ensiformis agglutinin (Con A) and with Ulex europaeus agglutinin-I (UEA-I). Succinylated wheat germ agglutinin (S-WGA) stained affected visceral cells and astrocytes and macrophages in the central nervous system. These results demonstrate that alpha-D-mannosyl and alpha-L-fucosyl residues, which bind Con A and UEA-I, respectively, are present in affected neurons. Furthermore, they revealed the affected non-neuronal cells and astrocytes contain complex carbohydrates with nonreducing terminal beta-N-acetylglucosamine, which binds S-WGA.

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Neurophysiological studies in GM1, gangliosidosis

Harden

Martinović

Pampiglione

1982

Ital J Neuro Sci

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Neurophysiological studies (EEG, ERG, VEP) have been carried out on 8 children with proven GM1 gangliosidosis (3 of Type I and 5 of Type II). All the EEGs were abnormal showing an increasing amount of irregular slow activity as the disease progressed. Around 2 to 3 years of age, Type II patients often showed a fluctuating 4-5 c/s rhythmic activity especially prominent in the temporal regions. Paroxysmal activity was not a conspicuous feature in any of the patients. The ERG was normal in all cases but the VEP was variably altered. The EEG/ERG/VEP findings in GM1 gangliosidosis differ from those seen in most other neurometabolic disorders of childhood.

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Pathology

Cited by 4 publications

References 77 publications

Progress in investigations of sphingolipidoses

Progress in investigations of sphingolipidoses

Lectin histochemistry of gangliosidosis

Neurophysiological studies in GM1, gangliosidosis

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