Abstract

Obejective: Reversible cerebral vasoconstriction syndrome (RCVS), an uncommon entity usually presenting as severe headache with or without neurological deficits, involves multifocal cerebral vasoconstriction, and may be associated with stroke. Multiple triggering etiologies are proposed, including ascent to high altitude. This case, triggered by high altitude, introduces a syndrome not as yet described in the neuropsychological literature, providing data from neuropsychological assessment obtained after resolution of vasoconstriction. Method: A healthy middle-aged woman experienced thunderclap headache while skiing at high altitude. She was evaluated locally and also en route to her final destination. Evaluation including neuroimaging was unremarkable; diagnosis of migraine headache was made. Two weeks later she presented to our medical center where neuroimaging showed bilateral (R > L) parieto-occipital infarctions. MR angiography and brain biopsy findings led to diagnosis of RCVS. After comprehensive rehabilitation, impaired motor and tactile functions showed recovery, but some visual symptoms remained; cognitive deficits were suspected. Neuropsychological evaluation was completed 18 months later. Results: Comprehensive neuropsychological testing was abnormal. Essentially intact performances were obtained in verbal intellectual, executive and other neurocognitive functions, with impairments identified in perceptual organization and visuospatial neurocognitive abilities, beyond those attributable to residual visual deficit alone. MRI findings (included) were compatible with neuropsychological test results. Conclusion(s): This case documents persistent neuropsychological deficits in an uncommon neurologic syndrome (RCVS) with rare circumstance of trigger by high altitude. The case shows neuroimaging findings remarkably similar to a published case of RCVS precipitated by high altitude ascent. Current case adds findings describing neuropsychological residuals, not previously reported.