Download PDF
CC BY-NC-ND 4.0 · Asian J Neurosurg 2021; 16(03): 549-553
DOI: 10.4103/ajns.AJNS_92_21
Original Article

Clinical and outcome analysis in head injury patients with fahr's disease

Rahul Singh
Department of Neurosurgery, Institute of Medical Sciences, Banaras Hindu University, Varanasi, Uttar Pradesh
,
Anurag Sahu
Department of Neurosurgery, Institute of Medical Sciences, Banaras Hindu University, Varanasi, Uttar Pradesh
,
Ramit Singh
Department of Neurosurgery, Institute of Medical Sciences, Banaras Hindu University, Varanasi, Uttar Pradesh
,
Kulwant Bhaikhel
Department of Neurosurgery, Institute of Medical Sciences, Banaras Hindu University, Varanasi, Uttar Pradesh
,
Ravi Prasad
Department of Neurosurgery, Institute of Medical Sciences, Banaras Hindu University, Varanasi, Uttar Pradesh
› Author Affiliations
› Further Information
Also available at
   Permissions and Reprints

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.

Key-words:

Clinical presentation - Fahr's disease - head injury - outcome analysis

Financial support and sponsorship

Nil.




Publication History

Received: 02 March 2021

Accepted: 30 May 2021

Article published online:
16 August 2022

© 2021. Asian Congress of Neurological Surgeons. This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commercial purposes, or adapted, remixed, transformed or built upon. (https://creativecommons.org/licenses/by-nc-nd/4.0/)

Thieme Medical and Scientific Publishers Pvt. Ltd.
A-12, 2nd Floor, Sector 2, Noida-201301 UP, India

 

  • References

  • 1 Sahin N, Solak A, Genc B, Kulu U. Fahr disease: Use of susceptibility-weighted imaging for diagnostic dilemma with magnetic resonance imaging. Quant Imaging Med Surg 2015;5:628-32.
  • 2 Saleem S, Aslam HM, Anwar M, Anwar S, Saleem M, Saleem A, et al. Fahr's syndrome: Literature review of current evidence. Orphanet J Rare Dis 2013;8:156.
  • 3 Yamada N, Hayashi T. Asymptomatic familial basal ganglia calcification with autosomal dominant inheritance: A family report. No To Hattatsu 2000;32:515-9.
  • 4 Malik R, Pandya VK, Naik D. FAHR disease: A rare neurodegenerative disorder. Indian J Radiol Imaging 2004;14:383-4.
  • 5 Fénelon G, Gray F, Paillard F, Thibierge M, Mahieux F, Guillani A. A prospective study of patients with CT detected pallidal calcifications. J Neurol Neurosurg Psychiatry 1993;56:622-5.
  • 6 Ooi HW, Er C, Hussain I, Kuthiah N, Meyyur Aravamudan V. Bilateral Basal Ganglia Calcification: Fahr's Disease. Cureus. 2019 Jun 1;11(6):e4797. doi: 10.7759/cureus.4797. PMID: 31396467; PMCID: PMC6679705.
  • 7 Shakibai SV, Johnson JP, Bourgeois JA. Paranoid delusions and cognitive impairment suggesting Fahr's disease. Psychosomatics 2005;46:569-72.
  • 8 Wu Z, Mittal S, Kish K, Yu Y, Hu J, Haacke EM. Identification of calcification with MRI using susceptibility-weighted imaging: A case study. J Magn Reson Imaging 2009;29:177-82.
  • 9 Kazis AD. Contribution of CT scan to the diagnosis of Fahr's syndrome. Acta Neurol Scand 1985;71:206-11.
  • 10 Lauterbach, E., 2005. Psychiatric management in neurological disease. Washington, DC: American Psychiatric Press. Available from: http://books.google.com.pk/books?id=sE1qlE751XcC. [Last assessed on 2021 Feb 02 ].
  • 11 König P. Psychopathological alterations in cases of symmetrical basal ganglia sclerosis. Biol Psychiatry 1989;25:459-68.
  • 12 Kendall B, Cavanagh N. Intracranial calcification in pediatric computed tomography. Neuroradiology 1986;28:324-30.
  • 13 Vles JS, Lodder J, van der Lugt PJ. Clinical significance of basal ganglia calcifications detected by ct (a retrospective study of 33 cases). Clin Neurol Neurosurg 1981;83:253-6.
  • 14 Weisman DC, Yaari R, Hansen LA, Thal LJ. Density of the brain, decline of the mind: An atypical case of Fahr disease. Arch Neurol 2007;64:756-7.
  • 15 Manyam BV. What is and what is not “Fahr' disease”. Parkinsonism Relat Disorder 2005;11:73-80.
  • 16 Asokan AG, D souza S, Jeganathan J, Pai S. Fahr's syndrome – An interesting case presentation. J Clin Diagn Res 2013;7:532-3.
  • 17 Pourshahid S, Salloum MN, Elfishawi M, Barakat M, Basith M. Fahr's disease: A differential to be considered for various neuropsychiatric presentations. Cureus 2018;10:e2304.
  • 18 Lam JS, Fong SY, Yiu GC, Wing YK. Fahr's disease: A differential diagnosis of frontal lobe syndrome. Hong Kong Med J 2007;13:75-7.
  • 19 Manyam BV, Walters AS, Narla KR. Bilateral striopalliododentate calcinosis: Clinical characteristics of patients seen in a registry. Mov Disord 2001;16:258-64.
  • 20 Manyam BV, Bhatt MH, Moore WD, Devleschoward AB, Anderson DR, Calne DB. Bilateral striopallidodentate calcinosis: Cerebrospinal fluid, imaging, and electrophysiological studies. Ann Neurol 1992;31:379-84.