Buradasınız

BİR MULTİFOKAL DİSTONİ OLGUSU

A PATIENT WITH MULTIFOCAL DYSTONIA

Journal Name:

Publication Year:

Keywords (Original Language):

Abstract (2. Language): 
Aim: Diagnosed with multifocal dystonia, the subjects without trauma, exposure to toxic sub¬stances, metabolic diseases or the history of chronic drug use were aimed to be discussed in light of literature. Case Report: A 62- year-old man was recruited to our clinic with the complaints of the pain on the right half of the neck, tenderness, contractions on both hands, more definite on the right. In his history, it was determined that the pain on the right half of the neck had been present for two years and the complaint of contraction on the right hand had just started after the right neck pain, and that contraction on the left hand had existed for one year. On his physical examination,a mass which could palpably be felt, in size of 1x1 cm, of neatly framed and tender was observed on the right half of the neck. On the neurologic examination, dystonia existed on both hands, more marked on the right, and tonus had increased on both upper extremities in the nature of rigidity. On the cervical MRI, definite protrusion on C5-6, left paramedian protrusion on C5-6, C6-7 and cord impression on C5-6 were present. Conclusion: What makes our case remarkable is timely correla¬tion between pain on right half of neck and beginning of dystonia on right upper extremity. Chronic pain could lead to movement disorder in the form of dystonia by disinhibitation of the silent spinal cord central pattern generator by causing hyperexitability of spinal interneurons.
Abstract (Original Language): 
Amaç: Travma, toksik maddeye maruziyet, metabolik hastalık ya da kronik ilaç kullanım öyküsü olmayan multifokal distonili hastanın literatür ışığında tartışılması amaçlandı. Olgu Sunumu: Altmış iki yaşında erkek hasta boynun sağ yarısında ağrı, hassasiyet, sağda belirgin her iki elde kasılma şikayetleri ile servisimize yatırıldı. Öyküde boynun sağ yarısındaki ağrının iki yıldır mevcut olduğu ve bu ağrının hemen ardından sağ elde kasılma şikayetinin başladığı, bir yıldır da sol elde kasılma şikayetinin mevcut olduğu tespit edildi. Genel fizik muayenede boynun sağ yarısında dokunmakla hassas 1x1 cm ebadında düzgün sınırlı ele gelen kitle gözlendi. Nörolojik muayenede sağda daha belirgin her iki elde distoni mevcuttu, tonus her iki üst ekstremitede rjidite şeklinde artmıştı. Servikal MRG'de C5-6'da belirgin C5-6, C6-7'de sol paramedial protrüzyon, C5-6 seviyesinde kord basısı mev¬cuttu. Sonuç: Olgumuzu asıl ilgi çekici kılan boyun sağ yarısındaki ağrı ve sağ üst ekstremitedeki dis-toninin ortaya çıkışı arasındaki zamansal korelasyondur. Kronik ağrı, spinal internöronların hiperek-sitabilitesine neden olarak sessiz durumdaki spinal kordun santral pattern jeneratörünün disinhibis-yonu ile distoni şeklinde hareket bozukluğuna yol açabilir.

REFERENCES

References: 

1. Geyer HL, Bressman SB. The diagnosis of dystonia. Lancet Neurol 2006 ; 5 :780-90.
2. Chuang C, Fahn S, Frutch SJ. The natural history and treatment of acquired hemidystonia: report of 33 cases and review of the literature. J Neurol Ne-urosurg Psychiatry 2002; 72: 59-67.

3. Bressman SB, Leon D, Brin MF. Idiopathic torsion dystonia among Ashkenazi Jews: evidence for autosomal dominant inheritance. Ann Neurol 1989; 26: 612-20.
4. Bressman SB. Dystonia genotypes, phenotypes and classification. Adv Neurol 2004; 94: 101-7.

5. Bressman SB, Leon D, Kramer PL. Dystonia in Ash-kenazi Jews: clinical characterization of a founder mutation. Ann Neurol 1994; 36: 771-7.
6. Edwards M, Wood N, Bhatia K. Unusual phenoty-pes in DYT1 dystonia: a report of five cases and a review of the literature. Mov disord 2003; 18: 706-11.
7. Hill MD, Kumar R, Lozano A, Tator CH, Ashby P, Lang AE. Syringomyelic dystonia and athetosis.
Mov Disord 1999; 14: 684-88.
8. Nogues MA, Leiguarda RC, Rivero AD, Salvat F, Manes F. Involuntary movements and abnormal spontaneous EMG activity in syringomyelia and syringobulbia. Neurology 1999; 52: 823-34.
9. Tan EK, Lo YL, Chan LL, See SJ, Hong A, Wong
MC. Cervical disc prolapse with cord compression presenting with choreoathetosis and dystonia. Neurology 2002; 58:661-2.
10. van de Beek WJ, Vein A, Hilgevoord AA, van Djk JG, van Hilten BJ. Neurophysiologic aspects of patients with generalized or multifocal tonic dys-tonia of reflex sympathetic dystrophy. J Clin Neurophysiol 2002; 19(1):77-83.
11. Dietz V. Spinal cord pattern generators for loco-motion. Clinical Neurophysiol 2003; 114: 1379-89.
12. Scholz, J, Brom DC, Youn DH, Mills CD, Kohno T, Suter MR, Moore KA et al. Blocking caspase activity prevents transsynaptic neuronal apoptosis and the loss of inhibition in lamina II of the dorsal horn af¬ter peripheral injury. J Neurosci 2005; 25(32):
7317-23.
13. van Hilten JJ, van de Beek WJ, Vein AA, van Dijk JG, Middelkoop HA. Clinical aspects of multifocal or generalized tonic dystonia in reflex sympathetic
dystrophy. Neurology 2001; 56 (12): 1762-5.

Thank you for copying data from http://www.arastirmax.com