Potential Hidden Cause of Dementia Discovered - Neuroscience News

Summary: Some patients diagnosed with behavioral-variant frontotemporal dementia (bv-FTD) whitethorn alternatively beryllium suffering from a cerebrospinal fluid leak that leads to encephalon sagging.

Source: Cedars Sinai Medical Center

A caller Cedars-Sinai survey suggests that immoderate patients diagnosed with behavioral-variant frontotemporal dementia (bvFTD)—an incurable information that robs patients of the quality to power their behaviour and header with regular living—may alternatively person a cerebrospinal fluid leak, which is often treatable.

Researchers accidental these findings, published successful the peer-reviewed journal Alzheimer’s & Dementia: Translational Research and Clinical Interventions, whitethorn constituent the mode to a cure.

“Many of these patients experience cognitive, behavioral and property changes truthful terrible that they are arrested oregon placed successful nursing homes,” said Wouter Schievink, MD, manager of the Cerebrospinal Fluid Leak and Microvascular Neurosurgery Program and prof of Neurosurgery astatine Cedars-Sinai.

“If they person behavioral-variant frontotemporal dementia with an chartless cause, past nary attraction is available. But our survey shows that patients with cerebrospinal fluid leaks tin beryllium cured if we tin find the root of the leak.”

Cerebrospinal fluid (CSF) circulates successful and astir the brain and spinal cord to assistance cushion them from injury. When this fluid leaks into the body, the encephalon tin sag, causing dementia symptoms. Schievink said galore patients with encephalon sagging—which tin beryllium detected done MRI—go undiagnosed, and helium advises clinicians to instrumentality a 2nd look astatine patients with telltale symptoms.

“A knowledgeable radiologist, neurosurgeon oregon neurologist should cheque the patient’s MRI again to marque definite determination is nary grounds for encephalon sagging,” Schievink said.

Clinicians tin besides inquire astir a past of terrible headaches that amended erstwhile the diligent lies down, important sleepiness adjacent aft capable nighttime sleep, and whether the diligent has ever been diagnosed with a Chiari encephalon malformation, a information successful which brain tissue extends into the spinal canal. Brain sagging, Schievink said, is often mistaken for a Chiari malformation.

Even erstwhile encephalon sagging is detected, the root of a CSF leak tin beryllium hard to locate. When the fluid leaks done a teardrop oregon cyst successful the surrounding membrane, it is disposable connected CT myelogram imaging with the assistance of opposition medium.

Schievink and his squad precocious discovered an further origin of CSF leak: the CSF-venous fistula. In these cases, the fluid leaks into a vein, making it hard to spot connected a regular CT myelogram. To observe these leaks, technicians indispensable usage a specialized CT scan and observe the opposition mean successful question arsenic it flows done the cerebrospinal fluid.

In this study, investigators utilized this imaging method connected 21 patients with encephalon sagging and symptoms of bvFTD, and they discovered CSF-venous fistulas successful 9 of those patients. All 9 patients had their fistulas surgically closed, and their encephalon sagging and accompanying symptoms were wholly reversed.

Brain sagging, Schievink said, is often mistaken for a Chiari malformation. Image is successful the nationalist domain

“This is simply a rapidly evolving tract of study, and advances successful imaging exertion person greatly improved our quality to observe sources of CSF leak, particularly CSF-venous fistula,” said Keith L. Black, MD, seat of the section of Neurosurgery and the Ruth and Lawrence Harvey Chair successful Neuroscience astatine Cedars-Sinai.

“This specialized imaging is not wide available, and this survey suggests the request for further probe to amended detection and cure rates for patients.”

The remaining 12 survey participants, whose leaks could not beryllium identified, were treated with nontargeted therapies designed to relieve encephalon sagging, specified arsenic implantable systems for infusing the diligent with CSF. However, lone 3 of these patients experienced alleviation from their symptoms.

“Great efforts request to beryllium made to amended the detection complaint of CSF leak successful these patients,” Schievink said.

“We person developed nontargeted treatments for patients wherever nary leak tin beryllium detected, but arsenic our survey shows, these treatments are overmuch little effectual than targeted, surgical correction of the leak.”

About this dementia probe news

Author: Press Office
Source: Cedars Sinai Medical Center
Contact: Press Office – Cedars Sinai Medical Center
Image: The representation is successful the nationalist domain

Original Research: Open access.
“The reversible impairment of behavioral variant frontotemporal encephalon sagging syndrome: Challenges and opportunities” by Wouter I. Schievink et al. Alzheimer’s & Dementia: Translational Research & Clinical Interventions

Abstract

The reversible impairment of behavioral variant frontotemporal encephalon sagging syndrome: Challenges and opportunities

Introduction

Due to nonaccomplishment of encephalon buoyancy, spontaneous spinal cerebrospinal fluid (CSF) leaks origin orthostatic headaches but besides tin origin symptoms indistinguishable from behavioral variant frontotemporal dementia (bvFTD) owed to terrible encephalon sagging (including the frontal and temporal lobes), arsenic visualized connected encephalon magnetic resonance imaging. However, the detection of these CSF leaks whitethorn necessitate specialized spinal imaging techniques, specified arsenic integer subtraction myelography (DSM).

Methods

We performed DSM successful the lateral decubitus presumption nether wide anesthesia successful 21 consecutive patients with frontotemporal dementia encephalon sagging syndrome (4 women and 17 men; mean property 56.2 years [range: 31–70 years]).

Results

Nine patients (42.8%) were recovered to person a CSF-venous fistula, a precocious discovered benignant of CSF leak that cannot beryllium detected connected accepted spinal imaging. All 9 patients underwent uneventful surgical ligation of the fistula. Complete oregon near-complete and sustained solution of bvFTD symptoms was obtained by each 9 patients, accompanied by reversal of encephalon sagging, but successful lone 3 (25.0%) of the 12 patients successful whom nary CSF-venous fistula could beryllium detected (P = 0.0011), and who were treated with non-targeted therapies.

Discussion

Concerns astir a spinal CSF leak should not beryllium dismissed successful patients with frontotemporal encephalon sagging syndrome, adjacent erstwhile accepted spinal imaging is normal. However, adjacent with this specialized imaging the root of the nonaccomplishment of spinal CSF remains elusive successful much than fractional of patients.