Clinical and Outcome Analysis in Head Injury Patients with Fahr's Disease

CONTEXT: Fahr's disease (FD) is a rare neurodegenerative disorder. Head injury in patients with FD is an uncommon occurrence. AIM: The aim is to evaluate clinical and outcome characteristics in traumatic head injury patients with FD. SETTINGS AND DESIGN: Retrospective cohort study. MATERIALS AND METHODS: 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. STATISTICAL ANALYSIS USED: Fisher's exact test and unpaired t-test were used. P < 0.05 was considered statistically significant. RESULTS: 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 (P < 0.05). Neuropsychiatric features (P = 0.0210) were significantly more in females suffering from FD. CONCLUSIONS: Neurological features in FD predominate over neuropsychiatric and extrapyramidal symptoms. FD does not affect outcome following head injury.