Objective: To present a rare case of progressive supra-nuclear gaze palsy (PSP) in a patient with idiopathic basal ganglia calcification in a patient with idiopathic basal ganglia calcification.

Background: Fahr’s disease is an infrequent inherited or sporadic neurological disorder characterised by anomalous calcium deposition in basal ganglia, thalamus, dentate nucleus, cerebral cortex, cerebellum, subcortical white matter and even hippocampus. Depending up on the area of predominant involvement, the patient may manifest most commonly as parkinsonism, movement disorders or behavioural disturbances. PSP phenotype in idiopathic fahr’s disease is rarely reported.

Method: Case report.

Results: A 61-year woman with a background history of diabetes and hypertension, presented with progressive worsening of slowness of activities and gait impairment for the past one year.  Her symptoms were gradually worsened and had recurrent falls. Subsequently she has developed left leg dystonia. On examination she was found to have a sub-cortical dementia, up-gaze restriction of her eye movements, generalized rigidity, moderate bradykinesia, resting tremors of all four limbs along with left leg dystonia. CT brain and MRI Brain revealed bilateral symmetrical basal ganglia and cerebellar calcification. She was worked up for all the secondary causes of fahrs disease and all were negative. She was diagnosed to have idiopathic sporadic fahrs disease. She was commenced on levo-dopa, following which she had mild symptomatic improvement.

Conclusion: We present a case of idiopathic basal ganglia calcification, presenting at an atypical age, with rare PSP phenotype.

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MDS Abstracts - https://www.mdsabstracts.org/abstract/progressive-supra-nuclear-palsy-phenotype-with-idiopathic-basal-ganglia-calcification/