A rare case of rapidly progressive subacute sclerosing panencephalitis with atypical radiological involvement

Year 2014, Volume: 6 Issue: 3, 1 - 4, 01.12.2014

Semih Ayta Begüm Şirin Koç Miraç Ayşen Çakmak H.nilgün Selçuk Duru Murat Elevli

Abstract

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

Keywords

subacute sclerosing panencephalitis, fulminant course, acute disseminated encephalomyelitis

Atipik radyolojik tutulumla giden hızlı ilerleyişli nadir bir subakut sklerozan panensefalit olgusu

Abstract

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.

Keywords

subakut sklerozan panensefalit, fulminan gidiş, akut disemine ensefalomiyelit

References

• Garg RK. Subacute sclerosing panencephalitis. J Neurol 2008; 255: 1861-1871.
• World Health Organization (2006) Global Advi- sory Committee on Vaccine Safety, 1–2 Decem- ber 2005. Wkly Epidemiol Rec 81: 15–19.
• Bellini WJ, Rota JS, Lowe LE, et al. Subacute sclerosing panencephalitis: more cases of this fatal disease are prevented by measles immu- nization than was previously recognized. J Infect Dis 2005; 192: 1686-1693.
• Sarkar N, Gulati S, Dar L, Broor S, Kalra V. Diagnostic dilemmas in fulminant subacute sclerosing panencephalitis (SSPE). Indian J Pediatr 2004; 71: 365-367.
• Dimova P, Bojinova V. Subacute sclerosing panencephalitis with atypical onset. Clinical, computed tomographic, and magnetic res- onance imaging correlations. J Child Neurol 2000; 15: 258-260.
• Goraya J, Marks H, Khurana D, Legido A, Melvin J Subacute sclerosing panencephalitis (SSPE) presenting as acute disseminated en- cephalomyelitis in a child. J Child Neurol 2009; 24: 899-903.
• Öztürk A, Gurses C, Baykan B, Gökyiğit A, Eraksoy M. Subacute sclerosing panencepha- litis: clinical and magnetic resonance imaging evaluation of 36 patients. J Child Neurol 2002; 17: 25-29.
• Aydın K, Okur O, Tatlı B, Sarwar SG, Ozturk C, Dilber C. Reduced gray matter volume in the frontotemporal cortex of patients with early subacute sclerosing panencephalitis. AJNR Am J Neuroradiol 2009; 30: 271-275.
• Malik MA, Saeed M, Qureshi AU, Ahmed N, Akram M.Predictors of clinical course of suba- cute sclerosing panencephalitis: experience at the Children’s Hospital, Lahore. J Coll Physi- cians Surg Pak. 2010; 20: 671-674.
• Herguner MO, Altunbasak S, Baytok V. Patients with acute, fulminant form of SSPE. Turk J Pediatr. 2007; 49: 422-425.
• Wight C, Jin L, Nelson CS, Cosby SL, Padfield CJ.Case report: an autopsy proven case of fulminant subacute sclerosing panencephalitis. Neuropathol Appl Neurobiol 2003; 29: 312- 320.
• Marjanovic BD, Stojanov LM, Zamurovic DR, Pasić SS, Kravljanac RM, Djordjevi MS. Ful- minant subacute sclerosing panencephalitis: two cases with atypical presentation. Pediatr Neurol 2003; 29: 63-65.
• Cece H, Tokay L, Yildiz S, Karakas O, Karakas E, Iscan A. Epidemiological findings and clinical and magnetic resonance presentations in sub- acute sclerosing panencephalitis. J Int Med Res 2011; 39: 594-602.
• Yaramiş A, Taşkesen M. Brainstem involvement in subacute sclerosing panencephalitis. Turk J Pediatr 2010; 52: 542-545.
• Sharma P, Singh D, Singh MK, Garg RK, Kohli N. Brainstem involvement in subacute scleros- ing panencephalitis. Neurol India 2011; 59: 273-275.
• Brismar J, Gascon GG, von Steyern KV, Bohlega S.Subacute sclerosing panencephalitis: evalu- ation with CT and MR. AJNR Am J Neuroradiol 1996;17: 761–772.
• Comert S, Vitrinel A, Gursu HA, Deniz NC, Akin Y.Subacute sclerosing panencephalitis present- ing as acute disseminated encephalomyelitis. Indian J Pediatr 2006; 73: 1119-1121.
• Honarmand S, Glaser CA, Chow E, et al. Suba- cute sclerosing panencephalitis in the differen- tial diagnosis of encephalitis. Neurology 2004; 63: 1489-1493.
• Kurt AN, Kurt A, Aydın M, Kabakuş N. Subakut sklerozan panensefalitli hastalarımızın demo- grafik/ailesel özellikleri ve klinik bulguları. Tür- kiye Klinikleri Pediatri Dergisi 2005; 14: 10-13.