Case report: Cervical spinal cord signal changes in a case of adult-onset subacute sclerosing panenchephalitis

Introduction:Vision loss can be a consequence of numerous disorders of eye and neural pathway conveying visual input to brain. A variety of conditions can affect visual pathway producing neurogenic vision loss. The presentation and course of vision loss depends on the site of involvement and underlying etiology. We conducted this unprecedented study to evaluate the characteristics and outcome of various diseases of the visual pathway.Materials and Methods:In this prospective cohort study, we evaluated 64 patients with neurogenic visual impairment. Ophthalmological causes were excluded in all of them. Their presentation, ophthalmological characteristics and investigation findings were recorded. These patients were followed up till 6 months.Results:Out of 69 patients evaluated, 5 were excluded as they had ophthalmological abnormalities. The remaining 64 cases (113 eyes) were enrolled. 54 cases were due to diseases of anterior visual pathway and rest 10 had cortical vision loss. The etiologic distribution is as follows: Isolated optic neuritis- 12 (19%), multiple sclerosis- 4 (6.3%), neuromyelitis optica- 5 (7.9%), tubercular meningitis- 15 (23.8%), non-arteritic ischemic optic neuropathy, ischemic optic neuropathy complicating cavernous sinus thrombosis, cryptococcal meningitis, malignant infiltration of optic nerve, Crouzon's syndrome, calvarial thickening and traumatic occipital gliosis- 1 (1.6%) case each, idiopathic intracranial hypertension, pituitary adenoma, acute disseminated encephalomyelitis, posterior reversible leukoencephalopathy- 3 (4.8%) cases each, cortical venous thrombosis 5 (7.9%), subacute scleroing panencephalitis- 4 (6.3%) cases.Conclusions:The diseases of anterior visual pathway were much more common than cortical vision loss. A majority of our patients had severe impairment of vision at presentation.

show abstract

“…described a similar case of SSPE in a young adult lady who had vision loss followed years later by myelopath. [ 19 ]…”

Section: Discussionmentioning

confidence: 99%

Neurogenic vision loss: Causes and outcome. An experience from a tertiary center in Northern India

Verma

Gupta

Chaudhari

2014

Journal of Neurosciences in Rural Practice

View full text Add to dashboard Cite

show abstract

“…Such DW restriction has been described to be indicative of fulminant SSPE in a few case reports. [10,11] Involvement of spinal cord in SSPE is also rare, described only in few case reports [12][13] as cervical cord involvement. In our case, thoracic cord was involved with abnormal signal in the anterior and lateral cord.…”

Section: Discussionmentioning

confidence: 99%

Atypical imaging and clinical presentation of fulminant subacute sclerosing panencephalitis

Manek

Gala

Kulkarni

2023

CRCR

View full text Add to dashboard Cite

Subacute sclerosing panencephalitis (SSPE) is a late progressive fatal central nervous system sequelae of measles infection. The diagnosis is made based on clinical and electroencephalography (EEG) findings and confirmed by elevated titers of anti-measles antibodies in cerebrospinal fluid. The usual clinical presentation is in the form of progressive behavioral change, cognitive decline, and myoclonic jerks with some cases presenting in an atypical manner in the form of ataxia, epilepsy, and stroke. EEG is quiet characteristic in the form of periodic discharges of slow wave complex. Magnetic resonance imaging (MRI) early in the disease is usually normal with abnormal scans showing focal leukodystrophy, predominantly in the posterior cerebral white matter, and development of cortical atrophy with disease progression. Cord involvement is not common in SSPE. We report a child with SSPE who had initial presentation with cerebellar ataxia, acute progression of encephalitis, and atypical EEG findings. MRI brain on the initial presentation showed very subtle and focal abnormality which later progressed to have disseminated brain lesions and dorsal cord myelitis and further in the disease course showed cerebral venous sinus thrombosis. This case report emphasizes that SSPE can have very atypical presentation with rapid deterioration and can mimic as acute encephalitis or demyelinating disease.

show abstract

“…Histopathological and immunological studies on autopsy cases have demonstrated the involvement of the spinal cord 13 14. A case of adult onset SSPE has been reported with cervical cord signal changes on MRI 15. Presence of spinal cord signal changes on MRI calls for extensive medical and laboratory diagnosis to rule out other diseases.…”

Section: Discussionmentioning

confidence: 99%

Subacute sclerosing panencephalitis presenting as neuromyelitis optica

Raut¹,

Singh²,

Garg³

et al. 2012

BMJ Case Reports

View full text Add to dashboard Cite

SUMMARYSubacute sclerosing panencephalitis (SSPE) is a slowly progressing inflammatory and degenerative disorder of the brain caused by a mutant measles virus. The diagnosis of SSPE is based on characteristic clinical and EEG findings ( periodic complexes) and demonstration of elevated antibody titres against measles in cerebrospinal fluid. SSPE can have atypical clinical features at the onset. The authors here report a case of a 3-year-old child who presented with vision loss followed 15 months later by quadriparesis with bladder involvement. These clinical features resembled that of neuromyelitis optica. However, as the disease progressed, appearance of myoclonic jerks, periodic discharges on EEG and positive cerebrospinal fluid serology for measles led to the final diagnosis of SSPE. BACKGROUND

show abstract

Case report: Cervical spinal cord signal changes in a case of adult-onset subacute sclerosing panenchephalitis

Cited by 5 publications

References 8 publications

Neurogenic vision loss: Causes and outcome. An experience from a tertiary center in Northern India

Neurogenic vision loss: Causes and outcome. An experience from a tertiary center in Northern India

Atypical imaging and clinical presentation of fulminant subacute sclerosing panencephalitis

Subacute sclerosing panencephalitis presenting as neuromyelitis optica

Contact Info

Product

Resources

About