Frontotemporal brain sagging syndrome: Craniospinal hypovolemia secondary to a T6-T7 cerebrospinal fluid-venous fistula.

BACKGROUND: The frontotemporal brain sagging syndrome (FTBSS) is defined as an insidious/progressive decline in behavior and executive functions, hypersomnolence, and orthostatic headaches attributed to cerebrospinal fluid (CSF) hypovolemia. Here, a T6 CSF-venous fistula (e.g., between the subarachnoid CSF and a paraspinal vein) resulted in a CSF leak responsible for craniospinal hypovolemia. CASE DESCRIPTION: A 56-year-old male started with orthostatic headaches and fatigue after scuba diving. His symptoms included progressive, vertigo, tinnitus, nausea, lack of judgment, inappropriate behavior, memory dysfunction, apathy, tremor, orofacial dyskinesia, dysarthria, dysphagia, and hypersomnolence. The lumbar puncture revealed an opening pressure of 0 cm H2O. Magnetic resonance imaging (MRI) findings included brain sagging, bilateral temporal lobe herniation, and pachymeningeal enhancement. The computed tomography (CT) myelogram showed a thoracic diverticulum and a CSF-venous leak at the T6-T7 level. Surgery, which comprised a T6-T7 laminotomy, allowed for dissecting, clipping, and ligating the diverticulum/fistula. The patient improved postoperatively (e.g., cognitive, behavioral, and brainstem symptoms). The follow-up MRI's showed the reversion of the sagging index/uncal herniation. CONCLUSION: The FTBSS should be considered in the differential diagnosis of an early onset frontotemporal dementia. Establishing the diagnosis and localizing the site of a spinal CSF/venous leak warrant both MRI and myelogram CT studies, to pinpoint the CSF leak site for proper surgical clipping/ligation of these thoracic diverticulum/CSF-venous leaks.

Child with temporal lobe hamartoma: A to Z images and a case report.

Gelastic seizure was first described by Trousseau in 1877 and comes from the Greek word gelos (laughs), as laughter is the main feature [1]. Normal laughter is a reactive emotional behaviour and motor action that involves the limbic system, hypothalamus, temporal cortex, and several regions of the brainstem. A female patient, six years old, left-handed, with gelastic seizures, uncontrolled despite being treated with two antiepileptic drugs at high doses, was treated. A simple axial tomography was done, where a hypodense lesion that shapes the inner table of the skull temporal level was observed; later, magnetic resonance imaging was requested, better characterising an intraxial lesion in the right second temporal gyrus cystic appearance.