Akut dissemine ensefalomiyelit (ADEM)

Akut dissemine ensefalomiyelit (ADEM), merkezi sinir sisteminin enflamatuar, demiyelinizan bir hastalığıdır. Çocukluk çağındaki ADEM çalışmalarında, olguların yaklaşık dörtte üçünde öncesinde viral hastalık veya aşılanma öyküsü bulunur. Tanı, klinik ve radyolojik bulgulara dayanarak konulur. Manyetik rezonans görüntüleme (MR), uygun bir incelemedir ve gri madde tutulumu olabilmekle birlikte, özellikle ak madde değişikliklerini gösterir. Derin gri maddenin, özellikle bazal gangliyonların tutulumu daha sıktır. Klinik yineleme olmaksızın, ADEM sonrası ilk birkaç ayda yeni MR lezyonlannın saptanması multipl skleroz'un erken tanısında yardımcı olabilir. Multipl skleroz'un tersine ADEM, genellikle uzun süreli seyirin iyi olduğu, monofazik bir hastalıktır. Eğer relapslann aynı akut, monofazik immün sürecin bir parçası olduğu düşünülüyorsa 'multifazik dissemine ensefalomiyelit' (MDEM) terimi kullanılır. Yüksek doz intravenöz metilprednizolon tedavide etkin bulunmuştur. Bununla basan sağlanamazsa immunglobülinler, plazmaferez veya sitotoksik ilaçlar kullanılabilir.

Acute disseminated encephalomyelitis (ADEM)

Acute disseminated encephalomyelitis (ADEM) is an inflammatory demyelinating disease of the central nervous system in studies of childhood ADEM. In studies of childhood, ADEM documented prodromal viral illness, or vaccination history is present in approximately three-quarters of the subjects. Diagnosis is based on clinical and radiological findings. Magnetic Resonance Imaging (MRI) of the brain is the investigation of choice and typically demonstrates white matter changes, though grey matter may be involved. Involvement of the deep grey matter, particularly basal ganglia, is more frequent. In the absence of clinical recurrence, new MRI lesions that appear beyond the first few months after ADEM may help in the early diagnosis of multiple sclerosis (MS). In contrast to MS, ADEM is usually a monophasic disorder with favorable long-term prognosis. If relapses are thought to represent part of the same acute monophasic immune process, the term 'multiphasic disseminated encephalomyelitis' (MDEM) is used. High-dose intravenous methylprednisolon has been found to be effective. If that fails, immunoglobulins, plasmapheresis, or cytotoxic drugs can be administered.

PDF

___

1) Stonehouse B, Gupte G, Wassmer E, Whitehouse WP. Acute disseminated encephalomyelitis: recognition in the hands of general paediatricians. Arch Dis Child 2003; 88: 122- 4.
2) Yeh EA, Collins A, Cohen ME, Duffner PK, Faden H. Pediatrics. Detection of coronavirus in the central nervous system of a child with acute disseminated encephalomyelitis. Pediatrics 2004; 113: 73- 6.
3) Alper G, Schor NF. Toward definition of acute disseminated encephalitis of childhood. Curr Opin Pediatr 2004;16: 637-40.
4) Leake JAD, Albani S, Kao AS, et al. Acute disseminated encephalomyelitis in childhood: Epidemiologic, clinical and laboratory features. Pediatr Infect Dis J 2004; 23: 756- 64.
5) Garg RK. Acute disseminated encephalomyelitis. Postgraduate Medical Journal 2003; 79:11- 7.
6) Murthy JM. Acute disseminated encephalomyelitis. Neurol India 2002; 50: 238- 43.
7) Thomas GS, Hussain IH. Acute disseminated encephalomyelitis: a report of six cases. Med J Malaysia. 2004; 59: 342-51.
8) Brinar VV, Poser CM, Basic S, Petelin Z. Sudden onset aphasic hemiplegia: an unusual manifestation of disseminated encephalomyelitis. Clin Neurol Neurosurg. 2004; 106: 187- 96.
9) Hynson JL, Kornberg AJ, Coleman LT, Shield L, Harvey AS, Kean MJ. Clinical and neuroradiologic features of acute disseminated encephalomyelitis in children. Neurology 2001; 56: 1308- 12.
10) Lidia V, Gabis MD, Panasci DJ, Andriola MR, Huang W. Acute disseminated encephalomyelitis: an MRI/MRS longitudinal study. Pediatr Neurol 2004; 30: 324 9.
11) Dale RC, Sousa C, Chong wk, Cox TCS, Harding B, Neville BGR. Acute disseminated encephalomyelitis, multiphasic disseminated encephalomyelitis and multiple sclerosis in children. Brain 2000; 123: 2407- 22.
12) Andreula CF, Angelo NM, Recchia L, Milella D. MRI in the diagnosis of acute disseminated encephalomyelitis. Int J Neuroradiol 1997; 3: 21- 34.
13) Honkaniemi J, Dastidar P, Kahara V, Haapasalo H. Delayed MR imaging changes in acute dissaminated encephalomyelitis. Am J Neuroradiol. 2001; 22: 1117- 24.
14) Mader I, Wolff M, Nagele T, Niemann G, Grodd W, Kuker W. MRI and proton MR spectroscopy in acute disseminated encephalomyelitis. Childs Nerv Syst 2005; 21: 566- 72.
15) Inglese M, Salvi F, Ianucci G, Mancardi GL, Mascalchi M, Filippi M. Magnetization transfer and diffusion tensor MR imaging of acute disseminated encephalomyelitis. Am J Neuroradiol 2002; 23: 267- 72.
16) Rajesh B, Preeti S, Vishali M, Mamta V. Acute disseminated encephalomyelitis. Indian J Pediatr 2004; 71: 1035- 8.
17) Tardieu M, Mikaeloff Y. What is acute disseminated encephalomyelitis (ADEM)? Eur J Paediatr Neurol 2004; 8: 239- 42.