Corticobasal degeneration syndrome with basal ganglia calcification: Fahr's disease as a corticobasal look-alike?

Fahr's disease is a rare disorder of slowly progressive cognitive, psychiatric, and motor decline associated with idiopathic basal ganglia calcification (IBGC) and widespread calcification in the brain and cerebellum. Acute presentation of IBGC is most often as a seizure disorder; however, we present a case of an acute IBCG presentation in which the cause of the deterioration was an aneurysmal subarachnoid hemorrhage.

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A rare consortium of functional neurological disorder and Fahr’s disease - a herald of extensive calcification

Asian Journal of Medical Sciences ◽

10.3126/ajms.v11i6.30632 ◽

2020 ◽

Vol 11 (6) ◽

pp. 138-141

Author(s):

Alka Chauhan ◽

Sunny Garg

Keyword(s):

Basal Ganglia ◽

Neurological Disorder ◽

Neuropsychiatric Symptoms ◽

Cerebral Microvessels ◽

Basal Ganglia Calcification ◽

Fahr’S Disease ◽

Normal Limits ◽

Fahr's Disease ◽

Neuropsychiatric Manifestations ◽

Functional Neurological Disorder

Fahr’s disease is also known as familial idiopathic basal ganglia calcification affecting the cerebral microvessels and presenting with numerous neuropsychiatric manifestations. Functional neurological disorder (FND), a pure neuropsychiatric phenomenon occurs rarely in Fahr’s disease. Herein, we have presented a case of 17 year old female presenting neuropsychiatric symptoms like pseudoseizure, left sensorineural deficit and mild headache for last 4 months. Mental status examination revealed impaired attention and concentration. Laboratory examinations were within normal limits except CT scan and MRI Brain which revealed bilateral and symmetrical basal ganglia calcification. This is a rare case of FND associated with Fahr’s Disease, never reported in the literature. This case highlights the need for evaluating the biological factors in causation of FND where psychological factors were not assessed.

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Fahr's Disease: A Case Report

Journal of Nepal Paediatric Society ◽

10.3126/jnps.v30i1.2459 ◽

1970 ◽

Vol 30 (1) ◽

pp. 44-45

Author(s):

Rahul Sinha ◽

Kirandeep Sodhi ◽

BM John ◽

Daljit Singh

Keyword(s):

Basal Ganglia ◽

Dentate Nucleus ◽

Seizure Control ◽

Subcortical White Matter ◽

Indian Literature ◽

Basal Ganglia Calcification ◽

Fahr’S Disease ◽

Unusual Condition ◽

Fahr's Disease ◽

Key Words Basal Ganglia

We report a case of Fahrs disease in a 15year old girl presented to us with focal seizure with secondary generalisation. Clinically there were no abnormal findings. The CT scan (brain) showed multiple symmetric and extensive calcifications in the basal ganglia, thalamus, and dentate nucleus, subcortical white matter of cerebral and cerebellar areas. Presently the child is under followup with good seizure control with carbamezpine. This rare case of idiopathic Fahr's disease in a child, which has never been reported in Indian literature has been brought out to highlight this unusual condition and its differentiation from the commoner Fahr's syndrome. Key words: Basal ganglia, calcification, Fahrs disease. DOI: 10.3126/jnps.v30i1.2459 Journal of Nepal Paediatric Society Vol.30(1) 2010 44-45

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