Giglio Mariateresa, Corriero Alberto, Perillo Teresa, Varrassi Giustino, Puntillo Filomena
Department of Interdisciplinary Medicine, University of Bari and Aldo Moro, 70124 Bari, Italy.
Pediatric Unit, Policlinico Hospital, 70124 Bari, Italy.
Children (Basel). 2024 Nov 21;11(12):1410. doi: 10.3390/children11121410.
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.
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.
The combined pharmacological approach resulted in successful management of the patient's neuropathic pain and other symptoms associated with PFS, improving his overall condition.
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.
后颅窝综合征(PFS),也称为小脑缄默综合征,约25%接受后颅窝髓母细胞瘤切除术的儿科患者会出现该综合征。其主要特征为缄默或言语减少/受损,可能还包括运动功能障碍(失用症、共济失调、肌张力减退)、核上性脑神经麻痹、神经认知改变和情绪不稳定等多种症状。通常需要长期的多学科康复治疗,恢复大约需要六个月时间,不过许多儿童会有长期的残留缺陷。儿科患者中很少报告与PFS相关的神经性疼痛,且其治疗证据有限。
本病例报告描述了一名10岁男孩,在髓母细胞瘤不完全切除后出现PFS。临床表现包括缄默、易怒、情绪不稳定、睡眠障碍以及C5水平的神经性疼痛。该患者接受了加巴喷丁、地西泮和巴氯芬联合治疗。
联合药物治疗成功控制了患者的神经性疼痛及其他与PFS相关的症状,改善了他的整体状况。
本病例突出了多模式药物治疗方案在治疗患有PFS的儿科患者神经性疼痛及相关症状方面的潜在有效性。需要进一步研究以探索针对这种罕见但具有挑战性的并发症的最佳治疗策略。