To describe Gerstmann syndrome as a presentation of an uncommon central nervous system tumor – Intracranial solitary fibrous tumor (ISFT).

Gerstmann syndrome is a neurobehavioral syndrome characterized by four cardinal symptoms: acalculia, agraphia, finger-toe agnosia, and dysgraphia. The syndrome is caused primarily by lesions at the confluence of parietal, temporal and occipital lobes, but also can involve middle frontal lobe of the dominant hemisphere. Documented inciting lesions include stroke, tumor, hemorrhage, arteriovenous malformations, and seizures. ISFT is a diagnostic challenge due to its resemblance to more common brain tumors like meningioma, with histopathology being the definitive diagnostic test.

We describe a case presenting with the Gerstmann syndrome tetrad secondary to underlying ISFT.

Retrospective chart review and literature search.

A 37-year-old male who presented to our tertiary center with blurred vision, “not being himself”, and “acting funny” for 3 weeks. On exam, he was found to have a right homonymous hemianopsia, grade II papilledema and demonstrated all four symptoms of Gerstmann syndrome – inability to perform simple calculations (acalculia), or identify his fingers (finger agnosia), could not distinguish his left side from right (left-right disorientation), nor write out his name (agraphia). Magnetic resonance imaging (MRI) of the brain showed an extra-axial, highly vascularized 6-centimeter mass compressing the left parietal lobe.

He underwent a complete resection of the mass. Postoperatively, he had gradual improvement with complete resolution of agraphia, acalculia, finger agnosia, and left-right disorientation within a week status post resection. Tumor pathology indicated hemangiopericytoma/intracranial solitary fibrous tumor. On follow-up, he reported improvement in visual symptoms and no recurrence of the Gerstmann tetrad.

This case enunciates the enigmatic tetrad of Gerstmann syndrome. Though classically described as a sequela of stroke, mass effect of tumor on the parietal lobe may produce the symptoms which can resolve following resection.