Fahr’s syndrome is a rare entity encountered in emergency medicine and characterized by a large clinical spectrum. It can be idiopathic ,known as Fahr’s disease and related to an autosomic genetic dominant transmission, or secondary to multiple causes.
A 40-year-old woman presented to the Emergency Department with a complaint of 7-day persistent headache and dizziness history. On medical history, there was no prior similar pisodes, no recent medication use or trauma. On clinical examination, the patient was awake, afebrile, with normal vital signs and neurological exam. !e electrocardiogram showed a prolonged QTc with regular sinusal activity. A calcemia of 1,1mmol/l was noticed on laboratory examination. The patient’s cranial computerized tomography image is shown on figure 1.
Bilateral striopallidodentate calcinosis ( BSPDC) is a rare condition characterized by massive symmetrical calcifications involving mainly the basal ganglia with subsequent neuronal loss (1). !is entity has been described in the elderly but also known as Fahr’s disease related to a chromosomic autosomic dominant mutation. !ese intracranial symmetric calcifications have also been described with other secondary conditions such as metabolic disorders ( hypocalcemia) , infectious or inflammatory situations, encephalitis or poisoning (2). Clinical presentation is heterogeneous: patients can be asymptomatic or present with a large panel of neuropsychiatric clinical findings (3).
Acute headache revealing Fahr’s syndrome in Emergency Department
01
Fahr’s Syndrome: a rare clinic-radiologic entity.Rajul Rastogi, AK Singh, UC Rastogi and al. Medical Journal Armed Forces India. 2011;67:159-161.
02
What is and what is not ‘ Fahr’s disease’ . Bala V Manyan. Parkinsonism and Related Disorders.2005;11:73-80.
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Fahr’s disease: A rare neurological disease. Dinesh Chandhari,Pushpendra nath renjen. APPOLO medicine 2015; 12: 148-151.
