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FAHR SYNDROME SECONDARY TO HYPOPARATHYROIDISM: CASE REPORT

RESUMO

CASE PRESENTATION:
A. J.M., 68 years old, history of falls associated with vertigo and syncope for a year. Also, report of behavioral change, visual hallucinations and aggressiveness for eight months, receiving a diagnosis of schizophrenia. No other comorbidities. At physical examination, he was oriented in time and space, but unattentive, with difficulty in understanding commands and bradykinesia. Globally preserved strength and sensitivity. Presence of bilateral dysmetry with ataxic gait.
In Computed Tomography (CT) and Magnetic Resonance (MR) of the skull the presence of gross calcifications in base nuclei, supra and infratentorial white substance and bridge. Normal CSF and laboratory tests with low total calcium (CA) corrected by albumin, reduced parathyroid (PTH) (value: 4), 25-hydroxyvitamin D and normal urinary CA. Normal serological and rheumatoid exams, abdominal and chest CT without special features. Cervical ultrasound with reduced thyroid size.
In the face of the clinic and examinations, the patient presented criteria for diagnosis of hypoparathyroidism and Fahr Syndrome. Calcium replacement and symptomatic medications were performed, maintaining a stable neurological and neuropsychiatric condition.
DISCUSSION:
Fahr Syndrome is a rare and intractable neurodegenerative condition. It is cured with neuropsychiatric, cognitive and other manifestations such as motor symptoms, ataxia and aphasia. Among its causes, we included hypoparathyroidism, pseudoparathyroidism, vasculitis and hereditary diseases. Hypoparathyroidism results from deficient production of PTH, resulting in inefficient mobilization of bone calcium and inadequate renal resorption, leading to ectopic calcifications in various tissues, which, when intracerebral, characterizes the syndrome, in addition to serum hypocalcemia.
The calcium deposit in the central nervous system starts in the blood vessel wall and perivascular space and extends slowly to involve the whole neurone. Progressive calcification generates tissue damage and deposits of minerals. There is a predilection for basal ganglia, also reaching other areas. There is no specific treatment for the Syndrome, and it is only symptomatic.
FINAL COMMENTS:
The Fahr Syndrome is rare, but an important differential diagnosis in cases of intracranial calcifications, considering the need to discover the primary etiology for adequate treatment and orientation on the neurodegenerative character of the disease.

Palavras Chave

Fahr, Hypoparathyroidism, Calcifications