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Infectious Optic Neuropathies
Published in Vivek Lal, A Clinical Approach to Neuro-Ophthalmic Disorders, 2023
Imran Rizvi, Ravindra Kumar Garg
SSPE is a progressive brain disorder that is caused by a persistent mutated measles virus infection. SSPE is characterized by rapid cognitive decline, periodic myoclonic jerks and raised antimeasles antibody titer in cerebrospinal fluid.16 Up to 50% of the SSPE patients have ocular complications.17 In some visual manifestations precedes neurological manifestations. Visual manifestations in SSPE are either because of the optic nerve, retina or the visual cortex involvement.17–19 Necrotizing retinitis is the most common ocular manifestation in SSPE. The optic nerve involvement can manifest as papilledema, papillitis and optic atrophy.17,18,20,21 Retinal changes may dominantly be present in macular region.22 The involvement of cortex manifest with cortical blindness (Box 8.1).
Infectious Disease
Published in John S. Axford, Chris A. O'Callaghan, Medicine for Finals and Beyond, 2023
Susanna J. Dunachie, Hanif Esmail, Ruth Corrigan, Maria Dudareva
Neurological complications include encephalitis (1 in 1000 measles cases), acute disseminated encephalomyelitis (ADEM), and the late complication of subacute sclerosing panencephalitis (SSPE). SSPE is a rare, fatal, demyelinating encephalopathy caused by the measles virus remaining latent within the CNS producing this rare encephalitis many years later.
Measles
Published in Firza Alexander Gronthoud, Practical Clinical Microbiology and Infectious Diseases, 2020
The most frequent complications include viral pneumonitis and otitis media, as well as diarrhoea. Measles infection often leads to a temporary reduction in immune responses in the few weeks following infection, which may increase the risk of severe secondary bacterial and viral infections. Tracheobronchitis (‘measles croup’) and pneumonia due to secondary bacterial infection are frequent complications of measles. The most frequent CNS complication of measles is an acute immune-mediated, post-measles encephalitis occurring within 2–30 days. A CSF analysis shows a lymphocytic pleocytosis and increased protein. Differential diagnosis consists of enterovirus, herpes simplex 1 and varicella zoster. Other CNS manifestations are a primary measles encephalitis, measles inclusion body encephalitis and subacute sclerosing panencephalitis (SSPE), which is a very rare but very severe complication, occurring in about 0.01% of cases. Cases of SSPE present a few years after measles infection with progressive neurocognitive symptoms, which in most cases lead to coma and death. The risk of SSPE is increased in children who acquire measles before the age of 1 year.
Bilateral Vision Loss and Visual Hallucinations in Subacute Sclerosing Panencephalitis: A Case Report
Published in Neuro-Ophthalmology, 2023
Ravi Uniyal, Ravindra Kumar Garg, Hardeep Singh Malhotra, Neeraj Kumar, Shweta Pandey, Imran Rizvi, Amita Jain, Nidhi Tejan, Rupesh Singh kirar
SSPE is caused by the persistent mutant measles virus infection of the brain. It presents with cognitive decline, myoclonus, and focal and generalised seizures in children. However, atypical presentations are also often observed.8–15 SSPE typically manifests many years after acute measles infection. The virus gets entry into the brain during the acute exanthematous phase.8 In the brain, many structural and genetic changes happen in the virus that cause characteristic uracil‐to‐cytosine transitions in the M gene. This mutant virus continues to replicate in the brain parenchyma. CD4 and CD8 T-cells mediated inflammatory response ensues against the virus; however, it is not able to effectively kill the virus. Some researchers hypothesise that there is cross-reactivity between the virus and myelin antigens, which causes demyelination secondary to the autoimmune phenomenon.8 Histopathologically, SSPE is characterised by panencephalitis with parenchymal and leptomeningeal perivascular cellular infiltration. Inclusion bodies are characteristically seen in neurons and oligodendrocytes. Death generally occurs within 1–3 years. In acute fulminant form of SSPE, death occur within 6 months.16
Macular Necrotizing Retinitis as a Presenting Feature of Atypical Fulminant SSPE: A Case Report
Published in Ocular Immunology and Inflammation, 2023
Amruta More, Jayanti Singh, Nitin Chandak, Sachin B. Shetty, Alok Sen
Atypical fulminant SSPE may have varied presentations including gait disturbance, visual loss, uncontrolled seizures, extrapyramidal symptoms, and unexplained stroke in young patients.13 Mahadevan et al had reported a case of fulminant SSPE caused by the D7 variant of measles virus in which the patient presented with hemiparesis without typical SSPE features and succumbed to the disease within 19 days of presentation.11 In a series reported by Herguner et al, all three cases of fulminant SSPE had presented with neurological signs and succumbed to the disease within 2 months of presentation.12 Kandadai et al had reported a case of fulminant SSPE in a 15-year-old boy who presented with acute onset gait ataxia and right-sided hemiparesis.14 Similar to our case, Sarkar et al had reported a patient with only visual symptoms due to retinitis for 2 months followed by rapid neurological worsening leading to a vegetative state in 10 days.8 Ophthalmic features as sole presentation are not commonly reported and it is important to be aware of such atypical presentations of fulminant SSPE.
Adult-onset subacute sclerosing panencephalitis presenting with tonic motor seizures
Published in International Journal of Neuroscience, 2021
Ozge Yagcioglu Yassa, Gulay Kenangil, Ayse Destina Yalcin
Subacute sclerosing panencephalitis (SSPE) is a degenerative disease of the brain caused by a persistent measles virus infection occurring mostly in childhood or early adolescence [1, 2]. It is characterized by cognitive and mental deterioration with prominent myoclonic jerks and poor prognosis. The diagnosis depends on the characteristic EEG pattern and presence of a high titre of Anti-Measles IgG in serum and CSF. The incidence of SSPE has markedly decreased in developed countries since the introduction of live attenuated measles vaccine [3]. Although the number of cases also decreased (0.461 per million in Turkey), it is still not a rarity for pediatric neurologists in developing countries such as Turkey and India [3, 4]. The incidence of SSPE in Istanbul (Turkey) was found as 2 per million and measles vaccine was found to be highly protective against SSPE [5].