Restless legs syndrome in a person with Fahr's disease.

نویسندگان

  • Esra E Okuyucu
  • Tugba Tunc
  • Sinem Karazincir
  • Taskin Duman
چکیده

B striato-pallido-dentate calcinosis (Fahr’s disease) is known as bilateral symmetric calcification, mainly of the basal ganglia and the dentate nucleus of the cerebellum.1 The clinical features can be varied. The diagnosis is established by CT or MRI of brain and ruling out calcium metabolism abnormalities and developmental defects. Restless legs syndrome (RLS) is a common neurological sensorimotor disorder that encompasses an idiopathic form of genetic or unknown origin and symptomatic forms are due to many causes. The current understanding of the pathophysiology of RLS suggests the involvement of iron metabolism and dopaminergic dysfunction.2 We present a patient with RLS associated with striopallidodentate calcification. A 52-year-old right-handed female patient presented with a 3-year history of episodic, uncomfortable feelings in her legs while sitting or lying down especially in the evening or at night. When she experienced these uncomfortable sensations, she urgently needed to move her legs. Because of these unacceptable leg movements, she had been unable to sleep at nights for 3 years. Her mother had a similar history, with problems and difficulty in sleeping at nights. Neurological and physical examinations were normal. There was no extrapyramidal soft sign and neuropsychiatric abnormalities. Routine blood and urine tests, thyroid hormone level, blood ferritin and iron level, folic acid and vitamin B12 levels, blood ceruloplasmin levels, blood and urine copper levels were normal. Serum calcium, phosphorus and parathormone levels were also normal. Cerebrospinal fluid examination disclosed normal protein and glucose content with no cells. A search for antinuclear antibodies, anticardiolipin antibodies, lupus anticoagulant, antiHIV, anti-Epstein-Barr Virus, and anti-cytomegalovirus antibodies were negative. The EEG and nerve conduction studies were normal. Sleep examination by polysomnography showed in the early part of the night there were jerks known as periodic limb movements (PLMS). Radiological and echographic screening and blood tests for occult neoplasm were negative. A CT showed bilateral calcifications in the basal ganglia, thalamus, caudate nucleus, centrum semiovale, and dentate nucleus of the cerebellum (Figure 1). On MRI, calcified lesions produce various signal intensities. The T2-weighted images showed slightly hyperintense lesions in the bilateral basal ganglia, thalamus, centrum semiovale, and hypointense lesions in the caudate nucleus. The T1-weighted images showed hyperintense lesions in the bilateral basal ganglia, thalamus, and caudate nucleus (Figures 2a & b). The diagnosis of RLS was made in view of her typical symptoms. She was prescribed cabergoline 2 mg to be taken at night. She has now experienced an improved situation, regarding both the symptoms of RLS and sleeplessness. Clinical Notes

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عنوان ژورنال:
  • Neurosciences

دوره 14 1  شماره 

صفحات  -

تاریخ انتشار 2009