Rasmussen syndrome (RS) is a rare encephalitis characterized by inflammation of one cerebral hemisphere that causes atrophy, neurological and cognitive impairment. We present a 42-year-old female patient, with no significant previous history and diagnosed with epilepsy since she was 24 years old, epileptic seizures are reported in different time windows with temporary cessation, but with constant reinstallations of acute and drug-resistant episodes. Neurological examination revealed hyperkinesia of the extremities, with persistent flexion and extension movements. The neuropsychological evaluation carried out with the MoCA test, Rey’s complex figure and Token test, shows severe cognitive impairment with psychiatric symptoms. In addition, electroencephalogram results show unilateral hemispheric slowing with unilateral focal onset and MRI evidence of generalized atrophy of the left hemisphere, elements supporting the diagnosis of late-onset RS. Finally, information is offered about the associated neuropsychological manifestations.
encephalitis
Encephalitis and rapidly progressive dementia due to probable Prion disease presenting with a non-convulsive status epilepticus. Case report and literature review. Encefalitis y demencia rápidamente progresiva por probable enfermedad priónica que se presenta con un estado epiléptico no convulsivo. Reporte de caso y revisión de la literatura.
Prion diseases are rare and rapidly progressive fatal neurological disorders characterized by abnormal folding of neuronal proteins.
The diagnosis is often challenging and relies on a high clinical suspicion, imagenological findings, electroencephalographic (EEG) patterns and cerebrospinal fluid (CSF) analysis.
We present a case of probable prion disease with an accelerated neurological decline and a non-convulsive status epilepticus (NCSE), which has only been described in a few cases worldwide, and seems to be associated with worse neurological outcomes and shorter survival time.
Clinical manifestations, treatment, and outcomes are shown below.
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Encefalitis autoinmune por anticuerpos anti Iglon5, reporte de caso clínico. Autoinmune encephalitis due to anti Iglon5 antibodies, clinical case report
Introduction: New clinical entities related to the discovery of autoantibodies either against intracellular antigens or against cell membrane surface receptors or synapses are being identified with increasing frequency. In the latter group, patients with apnea have been described in recent years, REM and NON-REM sleep behavior disorder, respiratory failure and presence in serum and CSF of antibodies against the LON5 antigen. These antibodies appear to alter the interaction with the cytoskeletal system of the neuron and induce accumulation of TAU, conditioning a wide clinical expression.
Clinical case: 76-year-old male patient with eight years evolution of symptoms before diagnosis. He had a mixture of respiratory symptoms, parasomnias, ataxia, movement disorder, autonomic symptoms, cognitive and mood disorder, and associated a chronic demyelinating polyneuropathy. The diagnosis was confirmed by detection of antibodies against IGLON5 in cerebrospinal fluid.
Conclusion: Anti-IGLON5 antibody encephalitis is a unique entity that challenges what we know about autoimmunity and neurodegeneration, leading to a diagnostic challenge due to the wide expression of symptoms and the possibility of improvement with early treatment.
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Discapacidad asociada a manifestaciones neurológicas de COVID-19. Disability associated with COVID-19’s neurological manifestations
Introduction: COVID-19 is mainly a respiratory illness, however, with an incidence in nearly a third of patients of neurological manifestations secondary to affection to the central or peripheral nervous system, used to be more frequent in severe illness, having a wide range of disability and mortality.
Clinical case: We present the clinical case of a 39 years old female ,previously healthy, with diagnosis of COVID-19, initiating her illness with encephalitis and status epilepticus, posteriorly longitudinally extensive myelitis and anoxic-ischemic encephalopathy, which, despite of opportune diagnosis and treatment, she had a poor outcome developing persistent vegetative state.
Conclusions: COVID-19’s neurological manifestations could lead to a high risk of disability and mortality, needing an opportune detection and treatment, with a high suspicion of encephalitis and status epilepticus in patients with decrease of alertness and focal motor symptoms.
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Encefalitis Autoinmune Anti-Receptor NMDA Reporte de Caso Clínico y Revisión de Literatura. Anti-NMDA Receptor Encephalitis Case Report And Review Of Literature
Anti-NMDA Antibody encephalitis is an autoimmune entity characterized by the presence of autoantibodies against NMDA receptors. It is important to have in mind this entity, because it can be misdiagnosed initially due to its symptoms erroneously attributed to a psychiatric disorder, and diagnosis and treatment may be delayed. In this case report we describe a young woman who entered with neurological – psychiatric symptoms that simulate an acute schizophrenia and finally was diagnosed of autoimmune encephalitis.
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Encefalitis Límbica Autoinmune Asociada a Anticuerpos LGI1: Presentación de un Caso y Revisión de la Bibliografía. Autoimmune Limbic Encephalitis Associated With LGI1 Antibodies: Case Report And Review Of The Literature
Introduction: Leucine rich glioma inactivated protein 1 (LGI1) antibody encephalitis is a rare disease characterized by subacute memory impairment, behavioral disorders and epileptic seizures. Even most cases have a good outcome, residual cognitive deficits are common. Case report: 76-year-old woman who started with acute onset generalized tonic – clonic seizures and subsequent impaired level of consciousness. Antiepileptic treatment was started with slight clinical improvement. In magnetic resonance imaging performed during admission left hippocampal hyperintensity was seen in T2 and T2-FLAIR sequences. As autoimmune limbic encephalitis was suspected, immunomodulatory treatment with intravenous corticosteroids and immunoglobulins was started with clinical improvement. Afterwards, anti –LGI1 antibodies were positive in cerebrospinal fluid testing. Conclusions: anti – LGI1 antibody related encephalitis can produce different neurological manifestations and diverse onset, even acute. Early immunomodulatory treatment is important to improve both clinical manifestations and long – term outcome.
