Rasmussen Encephalitis: A Rare Neurological Disorder

A case of Rasmussen encephalitis in a 8 year old child is reported here who presented with recurrent focal seizures and progressive weakness of left half of body. EEG showed electrical features of epilepsy. MRI brain showed cortical atrophy of one brain hemisphere. On the basis of clinical and radiological evidence, diagnosis of Rasmussen encephalitis was made which is a rare neurological disorder of childhood.

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Ipsilateral motor evoked potentials in a patient with unihemispheric cortical atrophy due to Rasmussen encephalitis

Neural Regeneration Research ◽

10.4103/1673-5374.250581 ◽

2019 ◽

Vol 14 (6) ◽

pp. 1025

Author(s):

Raffaele Nardone ◽

PatrickB Langthaler ◽

Andrea Orioli ◽

Viviana Versace ◽

GiudittaIlaria Scarano ◽

...

Keyword(s):

Evoked Potentials ◽

Motor Evoked Potentials ◽

Cortical Atrophy ◽

Rasmussen Encephalitis

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New Onset Focal Seizure Following COVID-19 Vaccination: Case Report

10.21203/rs.3.rs-975438/v1 ◽

2021 ◽

Author(s):

Chun Seng Phua ◽

Shalini Bhaskar ◽

Azman Ali Raymond

Keyword(s):

Case Report ◽

Side Effects ◽

Focal Seizure ◽

Seizure Threshold ◽

Hippocampal Atrophy ◽

Case Presentation ◽

Mri Brain ◽

Focal Seizures ◽

Patient’S History ◽

New Onset

Abstract IntroductionAs more novel COVID-19 vaccines are being rolled out in a frantic pace globally, any complication that might be related to COVID-19 vaccines should be highlighted, especially since COVID-19 vaccines are relatively new, and side effects may yet to be fully elucidated. We report a case of a healthy 18-year-old male who presented with new onset focal seizures 5 days after receiving 1st dose of Oxford/AstraZeneca COVID-19 vaccine. Case PresentationThe patient was treated with intravenous phenytoin and oral levetiracetam 250mg twice daily with no further events. There was no documented fever. CT venogram and EEG were unremarkable. MRI brain revealed generalised atrophy including mild bilateral hippocampal atrophy with no evidence of sclerosis. There was no predilection for seizures identified from the patient’s history. The patient was discharged the following day on levetiracetam and advised to proceed with the 2nd dose of Oxford/AstraZeneca COVID-19 vaccination in 3 months’ time.ConclusionSeizures following COVID-19 vaccination have only been reported in a handful of cases. COVID-19 vaccination could lower seizure threshold, or unmask an underlying predisposition for epilepsy. As most COVID-19 vaccines worldwide are given in 2 doses, clinicians should consider maintaining patients on anti-seizure drugs if vaccination was thought to be a provoking factor.

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Cortical atrophy and air encephalography: a clinical and radiological study

Psychological Medicine ◽

10.1017/s0033291700049679 ◽

1973 ◽

Vol 3 (3) ◽

pp. 374-378 ◽

Cited By ~ 20

Author(s):

A. H. Mann

Keyword(s):

Lateral Ventricle ◽

Poor Prognosis ◽

The Body ◽

Cortical Atrophy ◽

Radiographic Examination ◽

Cortical Surface ◽

Radiological Study ◽

Radiological Evidence ◽

Air Encephalography

SYNOPSISForty-nine patients with radiological evidence of cortical atrophy were followed up five to 10 years after their radiographic examination. The survivors were examined personally. The original radiographs were then reassessed to establish features indicating a good and poor prognosis. Dimensions of the body of the lateral ventricle distinguished good and poor prognosis, whereas the appearance of the cortical surface did not.

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MRI brain volumetry in Rasmussen encephalitis: The fate of affected and “unaffected” hemispheres

Neurology ◽

10.1212/01.wnl.0000152895.23010.52 ◽

2005 ◽

Vol 64 (5) ◽

pp. 885-887 ◽

Cited By ~ 20

Author(s):

S. Larionov ◽

R. König ◽

H. Urbach ◽

R. Sassen ◽

C. E. Elger ◽

...

Keyword(s):

Rasmussen Encephalitis ◽

Mri Brain ◽

Brain Volumetry

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Hereditary spastic paraplegia-a differential for spastic paraplegia in children

International Journal of Contemporary Pediatrics ◽

10.18203/2349-3291.ijcp20205526 ◽

2020 ◽

Vol 8 (1) ◽

pp. 182

Author(s):

Niharika Shetty ◽

Sahana Devadas ◽

Mallesh Kariappa

Keyword(s):

Genetic Analysis ◽

Clinical Examination ◽

Neurodegenerative Disorders ◽

Hereditary Spastic Paraplegia ◽

Adult Life ◽

Lower Limbs ◽

Spastic Paraplegia ◽

Mri Brain ◽

Progressive Weakness ◽

Rule Out

Hereditary spastic paraplegia (HSP) is a group of neurodegenerative disorders with familial origin mainly affecting the lower limbs. It has a prevalence of 3-10/100,000. The diagnosis is by symptomatology, clinical examination and neuroimaging to rule out other causes of spastic paraplegia. The diagnosis is confirmed by genetic analysis. Since this disorder is slowly progressive with nonspecific MRI brain findings the diagnosis can be delayed with delay in the start of rehabilitation measures. This disorder is usually diagnosed in the adult life and the literature has very few cases of paediatric HSP and hence we are reporting a case of HSP. Here we present the case of two siblings who presented to us with progressive weakness of both the lower limbs.

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Jansky-Bielschowsky syndrome, a lysosomal disease: First diagnosed cash in Oman

Proceedings, annual meeting, Electron Microscopy Society of America ◽

10.1017/s0424820100123568 ◽

1992 ◽

Vol 50 (1) ◽

pp. 632-633

Author(s):

Line Buhl ◽

David Muirhead

Keyword(s):

Mental Retardation ◽

Clinical Picture ◽

Neurological Disorder ◽

Clinical Presentation ◽

Neuronal Ceroid Lipofuscinosis ◽

Infantile Form ◽

Lysosomal Disease ◽

Juvenile Form ◽

Progressive Encephalopathy ◽

Lysosomal Diseases

There are four lysosomal diseases of which the neuronal ceroid lipofuscinosis is the rarest. The clinical presentation and their characteric abnormal ultrastructure subdivide them into four types. These are known as the Infantile form (Santavuori-Haltia), Late infantile form (Jansky-Bielschowsky), Juvenile form (Batten-Spielmeyer-Voght) and the Adult form (Kuph's).An 8 year old Omani girl presented wth myclonic jerks since the age of 4 years, with progressive encephalopathy, mental retardation, ataxia and loss of vision. An ophthalmoscopy was performed followed by rectal suction biopsies (fig. 1). A previous sibling had died of an undiagnosed neurological disorder with a similar clinical picture.

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