What is the treatment for brain sagging (cerebellar ptosis)?

From the Guidelines

The treatment for brain sagging (cerebellar ptosis) primarily involves epidural blood patching, which seals cerebrospinal fluid (CSF) leaks causing the condition. This approach is supported by the most recent and highest quality study, which highlights the importance of imaging in diagnosing intracranial hypotension and localizing the source of the leak for targeted therapy 1. The study notes that the goals of imaging are two-fold: to confirm the diagnosis of intracranial hypotension and to localize the source of the leak for targeted therapy such as epidural blood patch, percutaneous fibrin glue treatment, endovascular venous fistula embolization, and surgical dural repair or venous fistula ligation.

Key Considerations

• Epidural blood patching involves injecting 15-20 ml of the patient's own blood into the epidural space, typically at the lumbar level.
• Multiple patches may be needed, with success rates around 30-50% for the first patch and increasing with subsequent treatments.
• Patients should maintain bed rest for 24-48 hours after the procedure to maximize effectiveness.
• Conservative measures include strict bed rest, increased fluid intake (2-3 liters daily), caffeine consumption (200-300 mg daily), and abdominal binders to temporarily relieve symptoms.

Alternative Approaches

• For patients who don't respond to blood patches, surgical intervention may be necessary to directly repair the dural tear causing the CSF leak.
• Medications like theophylline (200-300 mg twice daily) or steroids may provide symptomatic relief but don't address the underlying cause.
• The pathophysiologic mechanism of headache symptoms and various neurological deficits in patients with intracranial hypotension is not well understood but likely multifactorial and may be attributed to compensatory venodilitation, blood volume expansion, and dural sinus stretching as the body attempts to maintain a stable intracranial volume in response to decreased CSF volume, as well as downward traction on the meninges, nerves, and brain parenchyma as the brain loses buoyancy and begins to sag in response to decreased CSF volume 1.

From the Research

Treatment for Brain Sagging (Cerebellar Ptosis)

The treatment for brain sagging, also known as cerebellar ptosis, typically depends on the underlying cause of the condition.

• In cases where the brain sagging is caused by a cerebrospinal fluid (CSF) leak, treatment may involve surgical repair of the leak, such as a laminotomy to dissect, clip, and ligate the diverticulum/fistula 2.
• Epidural blood patch is considered a first-line treatment for brain sagging dementia (BSD) caused by spontaneous intracranial hypotension (SIH) 3, 4.
• Dural reduction surgery may also be a treatment option for frontotemporal brain sagging syndrome, particularly in cases where the dura is extremely thin and incompetent 5.
• The goal of treatment is to relieve symptoms, such as cognitive and behavioral changes, and to prevent further progression of the condition.
• With treatment, many patients experience partial or complete resolution of their symptoms, highlighting the importance of early diagnosis and treatment 3, 4, 6.

Diagnostic Considerations

• Diagnosis of brain sagging dementia (BSD) should be based on clinical signs and symptoms, as well as radiologic findings, including brain sagging and absence of frontotemporal atrophy 3.
• CSF leak can be identified with myelography and digital subtraction myelography 3.
• Magnetic resonance imaging (MRI) and computed tomography (CT) myelogram can help pinpoint the site of a spinal CSF/venous leak, allowing for proper surgical clipping/ligation of thoracic diverticulum/CSF-venous leaks 2.

Outcome and Prognosis

• Early diagnosis and treatment of brain sagging dementia (BSD) are crucial to prevent further progression of the condition and to improve outcomes 3, 6.
• With treatment, 81% of patients experienced partial and 67% complete resolution of their symptoms 3.
• Recognizing brain sagging dementia (BSD) as a treatable cause of dementia is essential to provide early treatment and to reverse cognitive and behavioral changes that may otherwise progress and fully impair the patient 3, 4, 6.