Abstract
Fahr syndrome is a rarely seen disease which is proceeded with calcinosis that consists as a result of Ca and P metabolism disorder at cerebellum thalamus and basal ganglia. It is characterized by neurodegenerative disorder. Deposition of Ca and several minerals can be seen at cranial ct. It has wide clinical course that indicates tetany to neuropsychiatric symptoms. Cranial ct is used at diagnosis of Fahr synd. treatment is usually symptomatic and includes correction of Ca metabolism disorder.Fahr syndrome must be considered at differential diagnosis of patients who has Ca metabolism disorder, suddenly developed neurological symptoms.
Keywords
References
- Hoque MA, Siddiqui MR, Arafat Y, Khan SU, Rahman KM, Mondol BA, Mohammad QD. Fahr's disease: a very rare cause of epilepsy. Mymensingh Med J 2010; 19: 127-129.
- Manyam BV. What is and what is not ‘Fahr’s disease’. Parkinsonism and Relat Disorders 2005; 11: 73-80.
- Andrea H Nemeth. GeneReviews™ [Internet]. Pagon RA, Bird TD, Dolan CR, et al., editors. Seattle (WA): University of Washington, Seattle; 1993
- Anderson J.R. Intracerebral calcifications in a case of SLE with nuerologic manifestations. Neuroradiology 1980; 19: 213-214.
- Illum F. Calcification of basal ganglia following carbon monoxide poisoning. Neurodiology 1980; 19: 213- 214.
- Harati Y, Jackson JA, Benjamin E. Adult onset idiopa- thic familial brain calcifications. Arch Intern Med 1984; 144: 2425-2427.
- Gescwind DH, Loginov M, Stern JM. Identification of a locus on chromosome 14Q for idiopathic basal ganglia calcification. Am J Genet 1999; 65: 764-772.
- Modrego PJ, Mojonero J. Fahr’s syndrome presenting with pure and progresive presenil dementia. Neurol Sci 2005; 26: 367-369.
- Shibayama H, Kobayashi H, Nakagawa M, Yamada K, Iwata H, Iwai K, Takeuchi T, Mu-Qune X, Ishihara R, Iwase S. Non Alzheimer, non-Pick dementia with Fahr’s syndrome. Clin Neuropathol 1992; 11: 237-250.
- Stelmasiak Z, Tarach JS. Idiopatic hypoparathyroidism with intracranial calcification and dominant skin ma- nifestions. Med Sci Monit 2000; 6: 145-150.
Abstract
Fahr sendromu serebellum, talamus ve bazal ganglionlarda kalsiyum ve fosfor metabolizma bozukluğu sonucu oluşan kalsinozisle seyreden nörodejeneratif bozukluklarla karakterize beyin tomografisi ile kalsiyum ve çeşitli minerallerin birikiminin gösterilebileceği nadir görülen bir hastalıktır. Tetaniden nöropsikiyatrik semptomlara kadar geniş bir klinik seyir gösterebilir. Tanısında Beyin tomografisi kullanılmakta olup tedavisi çoğu zaman semptomatik ve kalsiyum metabolizma bozukluğunun düzeltilmesi şeklindedir.Ani nöropsikiyatrik semptomlar görülen, kalsiyum metabolizma bozukluğu olup, sebebi anlaşılamayan nörolojik semptomlar gelişen hastalarda Fahr sendromu ayırıcı tanıda mutlaka hatırlanması gerekmektedir.
Keywords
References
- Hoque MA, Siddiqui MR, Arafat Y, Khan SU, Rahman KM, Mondol BA, Mohammad QD. Fahr's disease: a very rare cause of epilepsy. Mymensingh Med J 2010; 19: 127-129.
- Manyam BV. What is and what is not ‘Fahr’s disease’. Parkinsonism and Relat Disorders 2005; 11: 73-80.
- Andrea H Nemeth. GeneReviews™ [Internet]. Pagon RA, Bird TD, Dolan CR, et al., editors. Seattle (WA): University of Washington, Seattle; 1993
- Anderson J.R. Intracerebral calcifications in a case of SLE with nuerologic manifestations. Neuroradiology 1980; 19: 213-214.
- Illum F. Calcification of basal ganglia following carbon monoxide poisoning. Neurodiology 1980; 19: 213- 214.
- Harati Y, Jackson JA, Benjamin E. Adult onset idiopa- thic familial brain calcifications. Arch Intern Med 1984; 144: 2425-2427.
- Gescwind DH, Loginov M, Stern JM. Identification of a locus on chromosome 14Q for idiopathic basal ganglia calcification. Am J Genet 1999; 65: 764-772.
- Modrego PJ, Mojonero J. Fahr’s syndrome presenting with pure and progresive presenil dementia. Neurol Sci 2005; 26: 367-369.
- Shibayama H, Kobayashi H, Nakagawa M, Yamada K, Iwata H, Iwai K, Takeuchi T, Mu-Qune X, Ishihara R, Iwase S. Non Alzheimer, non-Pick dementia with Fahr’s syndrome. Clin Neuropathol 1992; 11: 237-250.
- Stelmasiak Z, Tarach JS. Idiopatic hypoparathyroidism with intracranial calcification and dominant skin ma- nifestions. Med Sci Monit 2000; 6: 145-150.
Details
| Primary Language | Turkish |
|---|---|
| Journal Section | Case Report |
| Authors | |
| Publication Date | September 1, 2012 |
| Published in Issue | Year 2012 Volume: 1 Issue: 3 |