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Pract Neurol. 2017 Aug;17(4):289-292. doi: 10.1136/practneurol-2016-001497. Epub 2017 Apr 22.

Hypoactive-hypoalert behaviour and thalamic hypometabolism due to intracranial hypotension.

Author information

1
Department of Neurology, Royal Victoria Hospital, Belfast, UK.
2
Department of Neuroradiology, Royal Victoria Hospital, Belfast, UK.
3
Department of Nuclear Medicine, Royal Victoria Hospital, Belfast, UK.
4
Department of Neuropsychology, Belfast Health and Social Care Trust, Belfast, UK.
5
Department of Neurology, Altnagelvin Hospital, Londonderry, UK.

Abstract

A 47-year-old man presented with a 9-year history of a hypoalert hypoactive behaviour syndrome, caused by the deep brain swelling variant of spontaneous intracranial hypotension. Along with apathy with retained cognition, he had stable ataxia, impaired upgaze and episodes of central apnoea. MRI brain showed a sagging brainstem, pointed ventricles and reduced angle between the vein of Galen and the straight sinus, but no meningeal enhancement or subdural collections. A dopamine transporter scan showed preganglionic dopamine receptor deficiency; a fluorodeoxy glucose positron emission tomography scan showed bilateral hypothalamic hypometabolism. This variant of spontaneous intracranial hypotension may alter deep brain functioning within the basal ganglia and thalamus, causing the hypoactive-hypoalert behaviour phenotype.

KEYWORDS:

Attention; Behavioural disorder; Clinical neurology; Neuroradialogy

PMID:
28433974
DOI:
10.1136/practneurol-2016-001497
[Indexed for MEDLINE]

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