Abstract
RATIONALE: Idiopathic normal pressure hydrocephalus (iNPH) occasionally co-exists with neurodegenerative disease, but its concurrence with Gerstmann syndrome (GS) has not been reported, leaving the reversibility of GS-like deficits after cerebrospinal fluid diversion unknown. PATIENT CONCERNS: A 77-year-old woman experienced a 1-year progressive decline in memory, object naming, and spatial orientation, eventually requiring institutional care. DIAGNOSES: Neurological examination revealed severe cognitive impairment (mini-mental state examination [MMSE] 4/30) with acalculia, agraphia, finger agnosia, and mild left-right disorientation, consistent with incomplete GS. Brain computed tomography demonstrated ventriculomegaly (Evans index 0.33), bilateral hippocampal atrophy, and a positive disproportionately enlarged subarachnoid space hydrocephalus sign, fulfilling Japanese iNPH criteria and suggesting comorbid Alzheimer's disease. INTERVENTIONS: In February 2024, a programmable ventriculoperitoneal shunt with a CODMAN CERTAS Plus valve was placed. OUTCOMES: At 1 month, MMSE improved to 9, and left-right disorientation, agraphia, and anomia resolved; finger agnosia recovered by 2 months, and acalculia partially improved by 3 months (MMSE 11). Serial imaging showed progressive resolution of the disproportionately enlarged subarachnoid space hydrocephalus sign, while ventricular size remained largely unchanged. No shunt-related complications occurred during the 3-month follow-up. LESSONS: Ventriculoperitoneal shunting can reverse higher cortical dysfunction - including GS-like symptoms - in iNPH even when imaging suggests concomitant Alzheimer's pathology. Recognition of such reversible neuropsychological signs may broaden surgical indications and improve patient outcomes.