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Question: What is Posterior Fossa Syndrome? Who does it affect and what can be done?
Answer:
Brain tumors are the most common solid tumour arising in childhood and are the second most common malignancy in children under 15 years of age, after leukemia. To a large extent, the complications following surgery to remove a brain tumour depend upon the tumour’s location within the brain and the surgical approach to remove it. Posterior Fossa Syndrome (also referred to as "Cerebellar Mutism Syndrome") is a transient complication arising after brain tumour removal in children. Dr. Harold Rekate, a pediatric neurosurgeon, first described it in 1985. It can occur in as many as 30% of patients and is more common in younger children and those with medulloblastoma, although surgery for any midline brain tumour involving the cerebellum (including ependymoma and astrocytoma) may be followed by this syndrome. Symptoms usually onset at 3 to 14 days after the operation and consist of decreased speech output (or mutism), inconsolable whining, mood swings, apathy, poor motor tone and head control. Other children may experience difficulty swallowing and diminished voluntary movements. Some display personality and cognitive changes. There appears to be a great deal of variability in the severity of the syndrome, with some children only mildly affected while some others are profoundly impaired. Speech always returns after a variable period of time and motor function and coordination gradually improve but the recovery may take up to a year.
The cerebellum has gradually been recognized as having an essential role in the complex neural systems for movement, balance, working memory, executive function, visual-spatial function, linguistic processing, attention, emotional modulation, and mood. Patients with posterior fossa lesions classically present with ataxia and incoordination. Cognitive, mood, and behavioral problems, however, are being described more often.
This is currently an active area of investigation and research in the fields of child psychology, psychiatry, speech and language pathology and neurosurgery. Our understanding of the cause is incomplete and current treatments are largely supportive until children regain the functions they have lost, with the assistance physiotherapy, occupational therapy and speech pathology.
A special thank you Dr. Adrianna Ranger, Pediatric Neurosurgeon at the London Health Sciences Centre for her time in answering this important questions for our readers. Dr. Ranger also volunteers with Brain Tumour Foundation of Canada as a member of our Professional Advisory Group, and a member of our Research Committee. Thank you again for all of your continued support.
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