Session Information
Date: Saturday, October 6, 2018
Session Title: Rare Genetic and Metabolic Diseases
Session Time: 1:45pm-3:15pm
Location: Hall 3FG
Objective: Two clinical cases of Fahr`s syndrome was presented.
Background: Fahr’s syndrome (FS) or secondary bilateral striatopallidodentate calcinosis is a rare neurological disorder characterized by bilateral symmetrical calcification of basal ganglia, cerebellar subcortical white matter, hippocampus and thalamus. It included a variety of neurological and psychiatric disturbances. FS is mostly associated with endocrinopathies, but can also be attributed to other etiologies such as infections, brain neoplasms or previous brain injury.
Methods: A 34 year old woman presented to the Neurological Department of the Odessa National Medical University Clinic with headache, painful muscle contraction of the limbs, depressed mood, anxiety, irregular sleep-wake syndrome. There is 5-year history of hypoparathyroidism and hypothyroidism is preceding. Neurological examination shows moderate bradykinetic Parkinsonian syndrome. CT revealed extensive bilateral symmetrical calcification of basal ganglia [figure1]. Patient improved with therapeutic doses of amantadine, venlafaxine and benzodiazepine.
Results: A 21-year-old woman complained about headache, anxiety, sleep disturbances. In the neurological status without pathology. CT of the brain: insignificant bilateral symmetrical calcification along both medial cerebral arteries and both cerebellum hemispheres. Patient improved with venlafaxine and benzo.
Conclusions: FS is a very rare disease and the pathophysiological mechanism of calcium deposits in extracellular and perivascular zones is unknown. Clinical picture could be poor with the prevalence of extrapyramidal or general symptoms. CT is the preferred method of determining the localization of lesions.
To cite this abstract in AMA style:
Y. Solodovnikova. Oligosymptomatic Fahr’s syndrome (2 clinical cases) [abstract]. Mov Disord. 2018; 33 (suppl 2). https://www.mdsabstracts.org/abstract/oligosymptomatic-fahrs-syndrome-2-clinical-cases/. Accessed October 5, 2024.« Back to 2018 International Congress
MDS Abstracts - https://www.mdsabstracts.org/abstract/oligosymptomatic-fahrs-syndrome-2-clinical-cases/