Singh Rahul, Sahu Anurag, Singh Ramit Chandra, Bhaikhel Kulwant Singh, Prasad Ravi Shankar
Department of Neurosurgery, Institute of Medical Sciences, Banaras Hindu University, Varanasi, Uttar Pradesh, India.
Asian J Neurosurg. 2021 Sep 14;16(3):549-553. doi: 10.4103/ajns.AJNS_92_21. eCollection 2021 Jul-Sep.
Fahr's disease (FD) is a rare neurodegenerative disorder. Head injury in patients with FD is an uncommon occurrence.
The aim is to evaluate clinical and outcome characteristics in traumatic head injury patients with FD.
Retrospective cohort study.
This retrospective cohort study includes 13 patients of FD presenting as head injury in neurosurgical emergency between September 2018 and February 2021. Each patient was evaluated in terms of demographic profile, Glasgow coma scale (GCS) at admission, severity of head injury, type of head injury, preexisting clinical features of FD, radiological findings, Glasgow outcome score (GOS), family history of FD, and biochemical abnormalities. Patients were also evaluated for dichotomized outcome (Good recovery: GOS 5-4 versus Poor recovery: GOS 1-3) and gender differences in FD presentation.
Fisher's exact test and unpaired -test were used. < 0.05 was considered statistically significant.
Neurological symptoms (69.2%), neuropsychiatric manifestations (46.1%) and extrapyramidal features (38.5%) were preexisting in these patients. Seizure (61.5%) was the most common neurological manifestation. Depression (23.1%) and anxiety disorder (15.4%) were common psychiatric disorders seen. Akathisia (23.1%) followed by tremor (15.4%) were predominant extrapyramidal presentations. On dichotomized outcome analysis, preexisting neurological, neuropsychiatric, and extrapyramidal manifestations due to FD were not associated significantly with outcome following head injury. GCS at admission, severity of head injury and pupillary changes were significantly associated with outcome ( < 0.05). Neuropsychiatric features ( = 0.0210) were significantly more in females suffering from FD.
Neurological features in FD predominate over neuropsychiatric and extrapyramidal symptoms. FD does not affect outcome following head injury.
法尔氏病（FD）是一种罕见的神经退行性疾病。FD患者发生头部损伤的情况并不常见。
评估FD创伤性颅脑损伤患者的临床及预后特征。
回顾性队列研究。
这项回顾性队列研究纳入了2018年9月至2021年2月期间因头部损伤在神经外科急诊就诊的13例FD患者。对每位患者进行了人口统计学资料、入院时格拉斯哥昏迷量表（GCS）、颅脑损伤严重程度、颅脑损伤类型、FD既往临床特征、影像学检查结果、格拉斯哥预后评分（GOS）、FD家族史及生化异常情况的评估。还对患者进行了二分法预后评估（良好恢复：GOS 5 - 4与不良恢复：GOS 1 - 3）以及FD表现的性别差异评估。
采用Fisher精确检验和非配对检验。P < 0.05被认为具有统计学意义。
这些患者中存在神经症状（69.2%）、神经精神表现（46.1%）和锥体外系特征（38.5%）。癫痫发作（61.5%）是最常见的神经表现。抑郁症（23.1%）和焦虑症（15.4%）是常见的精神疾病。静坐不能（23.1%）其次是震颤（15.4%）是主要的锥体外系表现。在二分法预后分析中，FD所致的既往神经、神经精神和锥体外系表现与颅脑损伤后的预后无显著相关性。入院时的GCS、颅脑损伤严重程度和瞳孔变化与预后显著相关（P < 0.05）。FD女性患者的神经精神特征（P = 0.0210）明显更多。
FD的神经特征比神经精神和锥体外系症状更突出。FD不影响颅脑损伤后的预后。
