Basal ganglia lesions in subacute sclerosing panencephalitis

Almeida, Kelson James; Brucki, Sonia Maria Dozzi; Duarte, Maria Irma Seixas; Pasqualucci, Carlos Augusto Gonçalves; Rosemberg, Sérgio; Nitrini, Ricardo

doi:10.1590/S1980-57642012DN06040014

ABSTRACT

The parieto-occipital region of the brain is the most frequently and severely affected in subacute sclerosing panencephalitis (SSPE). The basal ganglia, cerebellum and corpus callosum are less commonly involved. We describe a patient with SSPE confirmed by neuropathology based on brain magnetic resonance imaging showing extensive basal ganglia involvement and no significant involvement of other cortical structures. Though rarely described in SSPE, clinicians should be aware of this involvement. SSPE should be kept in mind when changes in basal ganglia signal are seen on brain magnetic resonance imaging with or without involvement of other regions of the human brain to avoid erroneous etiological diagnosis of other pathologies causing rapidly progressive dementia.

Key words: subacute sclerosing panencephalitis; measles; magnetic resonance imaging

RESUMO

A região parietooccipital é mais frequente e gravemente acometida na panencefalite esclerosante subaguda (PEESA). Os gânglios da base, cerebelo e corpo caloso são menos envolvidos. Descrevemos um paciente com PEESA confirmada por neuropatologia com imagens de ressonância magnética (RNM) evidenciando acometimento extenso dos gânglios da base sem envolvimento de outras estruturas corticais. Embora raramente descritas nesta doença, deve-se ficar atento para tal acometimento e PEESA deve ser lembrada quando alterações de sinal nos gânglios da base são vistas na RNM com ou sem acometimento de outras regiões do cérebro a fim de evitar outros diagnósticos etiológicos errôneos de patologias que cursam com demência rapidamente progressiva.

Palavras-chave: panencefalite esclerosante subaguda; sarampo; ressonância magnética

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REFERENCES

Heath CA, Smith C, Davenport R, Donnan GA. Progressive cognitive decline and myoclonus in a young woman: clinicopathological conference at the Edinburgh Advanced Neurology Course, 2007. Pract Neurol 2008;8:296-330.
Garg RK, Anuradha HK, Varma R, Singh MK, Sharma PK. Initial clinical and radiological findings in patients with SSPE: are they predictive of neurological outcome after 6 months of follow-up? J Clin Neurosci 2011;18:1458-1462.
Satishchandra P, Sinha S. Relevance of neuroimaging in the diagnosis and management of tropical neurologic disorders. Neuroimaging Clin N Am 2011;21:737-756.
Fabian VA, Lee HY, Keith-Rokosh JL, de Souza JL, Stewart-Wynne E. A 22-year-old Australian woman with atypical subacute sclerosing panencephalitis diagnosed at postmortem. J Clin Neurosci 2010;17:1192-1194.
Gutierrez J, Issacson RS, Koppel BS. Subacute sclerosing panencephalitis: an update. Dev Med Child Neurol 2010;52:901-907.
Malhotra HS, Garg RK, Naphade P. Cluster of partial motor seizures heralding the onset of hemimyoclonic subacute sclerosing panencephalitis. Mov Disord 2012; 27:958-959.
Garg RK. Subacute sclerosing panencephalitis. J Neurol 2008; 255: 1861-1871.
Schneider-Schaulies J, Meulen V, Schneider-Schaulies S. Measles infection of the central nervous system. J Neurovirol 2003;9:247-252.
Abuhandan M, Cece H, Calik M, Karakas E, Dogan F, Karakas O. An Evaluation of Subacute Sclerosing Panencephalitis Patients with Diffusion-Weighted Magnetic Resonance Imaging. Clin Neuroradiol 2012; 12, DOI: 10.1007/s00062-012-0163-0.
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;29:312-316.
Kravljanac R, Jovic N, Djuric M, Nikolic L. Epilepsia partialis continua in children with fulminant subacute sclerosing panencephalitis. Neurol Sci 2011; 32:1007-1012.
H Cece, L Tokay, S Yildiz, Karakas O, Karakas E, Iscan A. Epidemiological Findings and Clinical and Magnetic Resonance Presentations in Subacute Sclerosing Panencephalitis. J Int Med Res 2011;39: 594-602.

Publication Dates

Publication in this collection
Oct-Dec 2012

History

Received
07 Sept 2012
Accepted
03 Nov 2012

This is an open-access article distributed under the terms of the Creative Commons Attribution License

[1] Heath CA, Smith C, Davenport R, Donnan GA. Progressive cognitive decline and myoclonus in a young woman: clinicopathological conference at the Edinburgh Advanced Neurology Course, 2007. Pract Neurol 2008;8:296-330.

[2] Garg RK, Anuradha HK, Varma R, Singh MK, Sharma PK. Initial clinical and radiological findings in patients with SSPE: are they predictive of neurological outcome after 6 months of follow-up? J Clin Neurosci 2011;18:1458-1462.

[3] Satishchandra P, Sinha S. Relevance of neuroimaging in the diagnosis and management of tropical neurologic disorders. Neuroimaging Clin N Am 2011;21:737-756.

[4] Fabian VA, Lee HY, Keith-Rokosh JL, de Souza JL, Stewart-Wynne E. A 22-year-old Australian woman with atypical subacute sclerosing panencephalitis diagnosed at postmortem. J Clin Neurosci 2010;17:1192-1194.

[5] Gutierrez J, Issacson RS, Koppel BS. Subacute sclerosing panencephalitis: an update. Dev Med Child Neurol 2010;52:901-907.

[6] Malhotra HS, Garg RK, Naphade P. Cluster of partial motor seizures heralding the onset of hemimyoclonic subacute sclerosing panencephalitis. Mov Disord 2012; 27:958-959.

[7] Garg RK. Subacute sclerosing panencephalitis. J Neurol 2008; 255: 1861-1871.

[8] Schneider-Schaulies J, Meulen V, Schneider-Schaulies S. Measles infection of the central nervous system. J Neurovirol 2003;9:247-252.

[9] Abuhandan M, Cece H, Calik M, Karakas E, Dogan F, Karakas O. An Evaluation of Subacute Sclerosing Panencephalitis Patients with Diffusion-Weighted Magnetic Resonance Imaging. Clin Neuroradiol 2012; 12, DOI: 10.1007/s00062-012-0163-0.

[10] 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;29:312-316.

[11] Kravljanac R, Jovic N, Djuric M, Nikolic L. Epilepsia partialis continua in children with fulminant subacute sclerosing panencephalitis. Neurol Sci 2011; 32:1007-1012.

[12] H Cece, L Tokay, S Yildiz, Karakas O, Karakas E, Iscan A. Epidemiological Findings and Clinical and Magnetic Resonance Presentations in Subacute Sclerosing Panencephalitis. J Int Med Res 2011;39: 594-602.