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.
- Clinical neurology
- Behavioural disorder
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Contributors SK wrote the first draft and contributed to the revisions.
PF drafted neuroimaging text and figures and revised drafts of the paper.
SH drafted neuroimaging text and figure and revised the paper.
WS provided neuropsychometry data and revised drafts of the paper.
MOM conceived the idea of publication, coordinated preparation, submission and revisions.
Competing interests None declared.
Provenance and peer review Not commissioned; externally peer reviewed. This paper was reviewed by Masud Husain, Oxford, UK.