Background: Posterior fossa syndrome (PFS), also known as cerebellar mutism syndrome, occurs in about 25% of pediatric patients undergoing resection of a posterior cranial fossa medulloblastoma. It is characterized primarily by mutism or reduced/impaired speech and may include variable symptoms such as motor dysfunction (apraxia, ataxia, hypotonia), supranuclear cranial nerve palsies, neurocognitive changes, and emotional lability. Long-term multidisciplinary rehabilitation is typically required, with recovery taking approximately six months, though many children experience long-term residual deficits. Neuropathic pain associated with PFS is rarely reported in pediatric patients, and evidence for its management is limited. Methods: This case report describes a 10-year-old boy who developed PFS following incomplete resection of a medulloblastoma. Clinical presentation included mutism, irritability, emotional lability, sleep disturbances, and neuropathic pain localized at the C5 level. The patient was treated with a combination of gabapentin, diazepam, and baclofen. Results: The combined pharmacological approach resulted in successful management of the patient’s neuropathic pain and other symptoms associated with PFS, improving his overall condition. Conclusions: This case highlights the potential effectiveness of a multimodal pharmacological regimen for treating neuropathic pain and associated symptoms in pediatric patients with PFS. Further research is needed to explore optimal treatment strategies for this rare but challenging complication.

A Rare Case of Posterior Fossa Syndrome Associated with Neuropathic Pain Successfully Treated with a Combination of Gabapentin, Diazepam and Baclofen-A Case Report and Literature Review

Giglio, Mariateresa
;
Corriero, Alberto;Perillo, Teresa;Puntillo, Filomena
2024-01-01

Abstract

Background: Posterior fossa syndrome (PFS), also known as cerebellar mutism syndrome, occurs in about 25% of pediatric patients undergoing resection of a posterior cranial fossa medulloblastoma. It is characterized primarily by mutism or reduced/impaired speech and may include variable symptoms such as motor dysfunction (apraxia, ataxia, hypotonia), supranuclear cranial nerve palsies, neurocognitive changes, and emotional lability. Long-term multidisciplinary rehabilitation is typically required, with recovery taking approximately six months, though many children experience long-term residual deficits. Neuropathic pain associated with PFS is rarely reported in pediatric patients, and evidence for its management is limited. Methods: This case report describes a 10-year-old boy who developed PFS following incomplete resection of a medulloblastoma. Clinical presentation included mutism, irritability, emotional lability, sleep disturbances, and neuropathic pain localized at the C5 level. The patient was treated with a combination of gabapentin, diazepam, and baclofen. Results: The combined pharmacological approach resulted in successful management of the patient’s neuropathic pain and other symptoms associated with PFS, improving his overall condition. Conclusions: This case highlights the potential effectiveness of a multimodal pharmacological regimen for treating neuropathic pain and associated symptoms in pediatric patients with PFS. Further research is needed to explore optimal treatment strategies for this rare but challenging complication.
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/11586/530660
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