The Janus-faced nature of Rasmussen’s Encephalitis

Rasmussen encephalitis (RE) is an inflammatory unilateral progressive medically refractory epilepsy associated with hemiparesis, cognitive dysfunction, and hemispheric atrophy. Here, we present 2 cases from our institution that demonstrate the dual nature of RE in 2 similarly aged children. Overall, 2 types of RE have been described: type 1 has a short prodromal phase and more explosive onset and type 2 has a longer prodromal of partial seizures followed by hemiparesis and atrophy. Younger patients are more likely to fit into the type 1 presentation and have been described as more likely to have dual pathology. Perhaps the patients with a more acute onset are more likely to have a dual pathology as is found in our 2 cases. We review the typical findings in RE and discuss current treatment options, highlighting new experimental treatments.
Source: Seminars in Pediatric Neurology - Category: Neurology Authors: Source Type: research

Related Links:

Conclusion: Post-herpetic anti-NMDAr AIE could arise several months after infection. Clinicians must be aware of this possibility, particularly if cognitive and/or psychiatric symptoms occurred after a remitting period. In our two cases, only rituximab was associated with clinical improvement.
Source: Frontiers in Neurology - Category: Neurology Source Type: research
The malformations of cortical development (most FCD) were the most common pathological type for children cases in China with drug ‐resistant seizures. It was speculated further that the FCD patients with shorter duration of epilepsy before surgery seem to have a higher ratio of being seizure‐free after surgery. AbstractObjectivesThe drug ‐resistant seizures are characterized by frequent and severe onset of seizures in childhood. There is only little literature had extensively explored the types of pathological brain damage in Chinese children cases. The present study aims to investigate the histopathologic findings a...
Source: Brain and Behavior - Category: Neurology Authors: Tags: ORIGINAL RESEARCH Source Type: research
Conclusions: Thirty-two AE patients were recruited over 18 months. Twenty-one viral controls, 10 NI controls, and five other autoimmune neurological disease controls (AOND) were also included in the analysis. Our study found that conventional markers: presence of CSF monocytosis, oligoclonal bands, anti-neuronal immunofluorescence, and magnetic resonance imaging (MRI) changes could be suggestive of AE, but these investigations were neither sensitive nor specific. Promising novel makers of autoimmune encephalitis were the CSF cytokines IL-21 and IP10 which may provide better delineation between viral infections and autoimmu...
Source: Frontiers in Neurology - Category: Neurology Source Type: research
Conclusion: Continuous epileptic spasms were associated with severe brain impairments in patients with electro-clinical syndromes; and required long hospital stays in patients with acute symptomatic causes. We suggest to include continuous epileptic spasms in the international classification of status epilepticus, as a special form. Further investigations are required to better recognize this condition, better understand the etiology, as well as to explore more effective treatments to improve outcomes.
Source: Frontiers in Neurology - Category: Neurology Source Type: research
Anti-GAD65 antibodies have been identified in both acute/subacute seizures (limbic encephalitis and extralimbic encephalitis) and chronic isolated epilepsy. The evidence of high serum titers and intrathecal synthesis play a fundamental role in diagnosis but poorly correlate with disease severity or response to therapies. It remains controversial whether anti-GAD65 Abs are the pathogenic entity or only serve as a surrogate marker for autoimmune disorders mediated by cytotoxic T cells. Unlike other immune-mediated epilepsy, although multiple combinations of therapeutics are used, the efficacy and prognosis of patients with G...
Source: Journal of Neuroimmunology - Category: Allergy & Immunology Authors: Tags: Review Article Source Type: research
.
Source: International Journal of Neuroscience - Category: Neuroscience Authors: Source Type: research
We report two adults infected with HIV presenting with encephalopathy and seizures. Case 1 had a monophasic encephalopathy with detection of NMDAR antibodies in the context of HIV CSF escape. There was a clinical response to immunotherapy and anti-retroviral therapy adjustment. Case 2 initially presented in non-convulsive status epilepticus associated with HIV CSF escape. He responded to treatment with anti-epileptic drugs and anti-retroviral therapy alteration, but had two further neurological relapses. NMDAR antibodies were detected during the relapses and a clinical response was observed following treatment with immunot...
Source: Journal of Neurology - Category: Neurology Source Type: research
.
Source: International Journal of Neuroscience - Category: Neuroscience Authors: Source Type: research
ConclusionsMost patients have autoimmune or limbic encephalitis and corresponding abnormalities on neuroimaging. Other presentations include peripheral nerve hyperexcitability or Morvan syndromes, cerebellar syndromes, behavioral and cognitive changes, and more rarely movement disorders. The most commonly associated malignancy was thymoma and suggests a role for thymoma screening in CASPR2-related diseases.
Source: Journal of Neurology - Category: Neurology Source Type: research
Conclusions1. Atypical seizures, for instance, seizures without EEG evolution, are not rare but likely to be overlooked. 2. FBDS is closely linked with epileptic seizures, revealing FBDS to be a part of epileptic attacks. 3. HMs could expand the spectrum of motor manifestations, overlapping with sleep disorders. 4. The high prevalence of these motor events might be due to the disrupted cortical-subcortical network, which is critical in motor control and sleep.
Source: Seizure - Category: Neurology Source Type: research
More News: Brain | Children | Encephalitis | Epilepsy | Neurology | Pathology | Pediatrics