Ischemic stroke in a young patient with Fahr's disease: a case report.

Chih-Sheng Yang,Chung-Ping Lo,Man-Chun Wu

doi:10.1186/s12883-016-0557-8

Chih-Sheng Yang, Chung-Ping Lo + Show 1 more

Open Access

https://doi.org/10.1186/s12883-016-0557-8

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Abstract

BackgroundFahr’s disease is a rare neurodegenerative disorder characterized by diffuse intracranial calcium deposition and associated cell loss mainly in bilateral basal ganglia and dentate nuclei of the cerebellum. Subarachnoid hemorrhage and epileptic syncope had been reported as acute presentation of Fahr’s disease. We here report a 36-year-old male Indonesian diagnosed as Fahr’s disease presenting with young-onset ischemic stroke.Case presentationA 36-year-old male Indonesian without prior systemic disease or neurologic disorder presented with young-onset ischemic stroke involving the right posterior limb of internal capsule. Brain computed tomography and magnetic resonance imaging demonstrated symmetric calcifications in bilateral basal ganglia, internal capsules, cerebellar dentate nuclei, thalami, cerebral white matter, which were all consistent with Fahr’s disease. The laboratory studies excluded the presence of other pathologic processes leading to secondary intracranial calcification. Other young stroke surveys were unremarkable. After medical treatment and sustained physical rehabilitation for 3 months, he recovered to carry out daily activities independently.ConclusionWe present ischemic stroke in a young patient with sporadic Fahr’s disease. The differentiation between Fahr’s disease and Fahr’s syndrome is specially highlighted when brain CT exhibits diffuse, symmetric calcifications in bilateral basal ganglia, thalami, cerebellar dentate nuclei and cerebral white matter. The association between nonarteriosclerotic vascular calcification and cerebrovascular disease is worth special attention and further investigation.

Highlights

Fahr’s disease is a rare neurodegenerative disorder characterized by diffuse intracranial calcium deposition and associated cell loss mainly in bilateral basal ganglia and dentate nuclei of the cerebellum
Fahr’s disease or idiopathic basal ganglia calcification (IBGC) is a rare neurodegenerative disorder characterized by diffuse, symmetric intracranial calcium deposition and associated cell loss mainly in bilateral basal ganglia and dentate nuclei of the cerebellum in the absence of other causes leading to secondary calcification
The treatment resulted in favorable functional outcome that was assessed by the modified Rankin Scale score of 1 and the Barthel Index score of 100

Summary

Conclusion

Fahr’s disease or idiopathic basal ganglia calcification is a rare neurologic disorder with variable clinical presentations and distinctive neuroradiological features. Brain CT, supplemented by MRI study exhibited symmetric calcifications in the bilateral basal ganglia, thalami, bilateral cerebral subcortical white matter, cerebellar dentate nuclei and deep cerebellar white matter, which were all consistent with Fahr’s disease. There have been no case reports demonstrating acute infarction in Fahr’s disease with positive MRI findings and describing the association with young-onset ischemic stroke. This is the first case report of Fahr’s disease presenting with ischemic stroke in Asian population. The differentiation between Fahr’s disease and Fahr’s syndrome is specially highlighted when brain CT shows diffuse, symmetric calcifications in bilateral basal ganglia, thalami, cerebellar dentate nuclei and cerebral white matter.

Background

Findings