Atipik radyolojik tutulumla giden hızlı ilerleyişli nadir bir subakut sklerozan panensefalit olgusu
Küçük Resim Yok
Tarih
2014
Dergi Başlığı
Dergi ISSN
Cilt Başlığı
Yayıncı
Maltepe Üniversitesi
Erişim Hakkı
Attribution-NonCommercial-NoDerivs 3.0 United States
info:eu-repo/semantics/openAccess
info:eu-repo/semantics/openAccess
Özet
Subakut sklerozan panensefalit (SSPE), kızamık infeksiyonundan yıllar sonra ortaya çıkan, mental kötüleşme, davranış değişiklikleri, miyoklonus ve nörolojik yıkımla seyreden bir hastalıktır. Biz akut konfüzyon ile başlayıp sıradışı radyolojik özellikler gösteren atipik bir SSPE olgusunu sunmayı amaçladık. Öncesinde sağlıklı olan 10 yaşında bir kız çocuğu bir haftadır devam eden baş ağrısı ve sürekli uyku hali ile başvurdu. Dokuz aylıkken kızamık enfeksiyonu öyküsü mevcuttu. Letarjik olup plantar refleks yanıtları bilateral ekstansördü. Sistemik muayene ve rutin biyokimyasal değerlendirmesi normaldi. Beyin manyetik rezonans görüntülemesinde (MRG) beyin sapından serebellar beyaz cevhere uzanan hiperintens lezyonlar görüldü. Rutin beyin omurilik sıvı incelemesi normal olup oligoklonal bantı pozitif, IgG indeksi> 0,7 idi. BOS kızamık antikor titreleri kuvvetli pozitif idi. Elektroensefalografisinde organizasyon bozukluğu ve jeneralize yavaş dalgaları görüldü. Intravenöz (IV) metilprednizolon 5 gün süre ile verildi. Anlamlı bir klinik yanıt alınamadığından IV immunoglobulin tedavisine geçildi. 2.haftada çekilen beyin MRG kontrolünde önceki lezyonlarında artış gözlendi. 4. haftada hasta ani kardiyak arrest nedeniyle kaybedildi. Sonuç: Akut fulminan seyir, beyin sapı ve serebellum tutuluşu SSPE için nadirdir. Baş ağrısı ve akut bilinç değişikliği ile başvuran, kranial görüntülemelerinde atipik bulgular saptanan olgularda subakut sklerozan panensefalit akılda tutulmalıdır.
Subacute sclerosing panencephalitis (SSPE) is a progressive disease characterized by mental-neurological deterioration and myoclonus, occurring after years of measles infection. Herein, we report an atypical SSPE case presented with acute confusion and headache which showed atypical radiological features. Case: A 10-year-old previously healthy girl was admitted with headache and constant sleepiness for a week. She had a history of measles infection at the age of nine months. She was lethargic and plantar reflexes were bilaterally indifferent. Systemic examination and routine biochemical evaluation were in normal limits. Brain magnetic resonance imaging (MRI) showed hyperintense lesions extending from brain stem to cerebellar white matter. Routine cerebrospinal fluid (CSF) examination was in normal limits with positive oligoclonal band and IgG index>0,7. CSF measles antibody titers were strongly positive. Her electroencephalography revealed disorganized back ground and generalized slow waves. Intravenous (IV) metilprednizolone was given for 5 days. Because of no significant clinical response, the treatment was switched to IV immunoglobuline. At the second week, following MRI showed extention of previous lesions. On the 4th week of presentation, myoclonus began, the patient developed sudden cardiac arrest and died. Conclusion: Acute fulminant course and involvement of brainstem and cerebellum is rare in SSPE. Differential diagnosis may be difficult from other acute confusional states. This case report seeks to draw attention to the neccessity of keeping the subacute sclerosing panencephalitis in mind while considering the differential diagnosis in patients with headache and acute altered mental state with atypical findings on the brain imaging
Subacute sclerosing panencephalitis (SSPE) is a progressive disease characterized by mental-neurological deterioration and myoclonus, occurring after years of measles infection. Herein, we report an atypical SSPE case presented with acute confusion and headache which showed atypical radiological features. Case: A 10-year-old previously healthy girl was admitted with headache and constant sleepiness for a week. She had a history of measles infection at the age of nine months. She was lethargic and plantar reflexes were bilaterally indifferent. Systemic examination and routine biochemical evaluation were in normal limits. Brain magnetic resonance imaging (MRI) showed hyperintense lesions extending from brain stem to cerebellar white matter. Routine cerebrospinal fluid (CSF) examination was in normal limits with positive oligoclonal band and IgG index>0,7. CSF measles antibody titers were strongly positive. Her electroencephalography revealed disorganized back ground and generalized slow waves. Intravenous (IV) metilprednizolone was given for 5 days. Because of no significant clinical response, the treatment was switched to IV immunoglobuline. At the second week, following MRI showed extention of previous lesions. On the 4th week of presentation, myoclonus began, the patient developed sudden cardiac arrest and died. Conclusion: Acute fulminant course and involvement of brainstem and cerebellum is rare in SSPE. Differential diagnosis may be difficult from other acute confusional states. This case report seeks to draw attention to the neccessity of keeping the subacute sclerosing panencephalitis in mind while considering the differential diagnosis in patients with headache and acute altered mental state with atypical findings on the brain imaging
Açıklama
Anahtar Kelimeler
subakut sklerozan panensefalit, fulminan gidiş, akut disemine ensefalomiyelit, subacute sclerosing panencephalitis, fulminant course, acute disseminated encephalomyelitis
Kaynak
Maltepe Tıp Dergisi
WoS Q Değeri
Scopus Q Değeri
Cilt
6
Sayı
3
Künye
Ayta, S., Koç, B. Ş., Çakmak, M. A., Selçuk Duru, H. N. ve Elevli, M. (2014). Atipik radyolojik tutulumla giden hızlı ilerleyişli nadir bir subakut sklerozan panensefalit olgusu / A rare case of rapidly progressive subacute sclerosing panencephalitis with atypical radiological involvement. Maltepe Tıp Dergisi. 6(3), s. 1-4.
