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December 1978

Neuroanatomical and Electroencephalographic Correlations in Sanfilippo Syndrome, Type A

Author Affiliations

From the Departments of Neurology (Drs Kriel and Hauser) and Pathology (Dr Posalaky), St Paul-Ramsey Hospital, and the Division of Pediatric Neurology (Dr Kriel) and the Departments of Neurology (Drs Hauser and Sung) and Laboratory Medicine and Pathology (Drs Sung and Posalaky), University of Minnesota Medical School, Minneapolis.

Arch Neurol. 1978;35(12):838-843. doi:10.1001/archneur.1978.00500360062013

• Waking and all-night-sleeping electroencephalographic recordings were obtained on a boy with Sanfilippo disease, type A. The most striking abnormalities were noted during sleep and included (1) lack of progression through normal sleep stages, (2) absence of vertex waves and normal sleep spindles, (3) inability to stage sleep by the usual criteria, and (4) an unusual alteration of low-amplitude (12 to 15 Hz) activity with generalized delta frequencies. Subsequently, neuropathological examination was performed. The electrophysiological phenomena may be correlated with severe cortical involvement.

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