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Massive Cerebral Calcifications (Fahr’s Disease) in a Patient with Systemic Lupus Erythematosus and No Major Neuropsychological Abnormality

Brain calcifications are uncommon findings in systemic lupus erythematosus ; if present they are usually accompanied by clinical signs of cerebral lupus [1]. Fahr’s disease refers to a rare syndrome characterized by abnormal symmetric and bilateral intracranial calcifications in certain areas of the brain. Most common sites are the basal ganglia and dentate nuclei. The condition is often asymptomatic and discovered on brain imaging [2]. Symptoms of the disorder may include deterioration of motor function, psychosis, dementia, seizures, headache, dysarthria, spasticity and spastic paralysis, eye impairments, and athetosis. Although the most common findings in Fahr’s disease are motor deficits, 40% of patients have primarily cognitive and other psychiatric symptoms. Fahr’s disease can also include symptoms characteristic of Parkinson’s disease such as tremor, muscle rigidity, mask-like facial appearance, shuffling gait and “pill-rolling” motion of the fingers. These generally occur later in the development of the disease. More common symptoms include dystonia and chorea. Age of onset is typically in the fifth or sixth decade of life, although it can occur at any time from childhood through adolescence to adulthood.

Fahr’s disease ; systemic lupus erythematosus (SLE) ; neuropsychiatric abnormalities ; insomnia

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