Rare Association of Lingual Hemiatrophy With Meningiomatosis: A Case Report

Hypoglossal nerve (cranial nerve XII) palsy is a rare condition, especially when isolated, and may arise from various etiologies, including tumors along its course. Among the rarest causes are meningiomas involving the hypoglossal canal. Meningiomatosis, the presence of multiple meningiomas throughout the central nervous system, is an uncommon entity that further complicates diagnosis. We report a case of a 64-year-old woman presenting with progressive right-sided tongue atrophy and deviation, without associated dysphonia or dysphagia. CT and MRI revealed fatty infiltration of the right hemitongue and demonstrated more than 10 intracranial meningiomas, including one involving the right hypoglossal canal, associated with bone remodeling. Imaging features, including the dural tail sign, homogenous enhancement, and adjacent bony changes, favored a meningioma over other differential diagnoses, such as schwannoma. The co-occurrence of meningiomatosis and isolated hypoglossal nerve palsy in a patient without neurofibromatosis type 2 is exceedingly rare. This case highlights the critical role of advanced imaging in localizing cranial neuropathies and underscores the need to consider meningiomatosis in the differential diagnosis of isolated hypoglossal nerve palsy.