Non epileptogenic Rasmussen`s encephalitis: a case report

Introduction: Rasmussen's encephalitis (RE) is a very rare chronic inflammatory unilateral encephalopathy with still unknown etiology. It predominantly affects children under the age of 10, but can also affect adolescents and adults. Current evidence on possible etiologies are divided in two main theories. The first one is based on brain inflammation caused by a reaction to a foreign antigen. The second one is based on an autoimmune disease that is limited to a single hemisphere of the brain. The common presentation is intractable and frequent focal motor seizures, often associated with progressive neurological decline, and progressive unilateral focal cortical atrophy. Progression of the inflammatory process in MRI is considered a good biomarker in RE. Objectives: Report a rare case of a female infant presenting atypical manifestations of uncommon symptoms without seizures caused by Rasmussen's encephalitis. Case Report: A ten-year-old right-handed female presented to the emergency department with sudden onset of abnormal involuntary movements of the right upper and lower limbs, face paresia of the same side and loss of attention without loss of consciousness. The involuntary contractions were brief, random, irregular, and worsened with activity, along with a positive milkmaid grip sign. She also displayed reduced movement speed and hyperactive biceps, triceps, patellar and ankle jerk reflexes. Associated with the motor abnormalities, the girl presented with a recent but progressive decrease in cognitive functions, which caused a decline in school performance and impaired reasoning, and affective blunting. She had no sign of previous neuropsychomotor development abnormality. She was taken to a quaternary hospital where a MRI and a CT of the brain were performed. MRI showed atrophy of the left cerebral hemisphere, predominantly in the operculum and head of the caudate nucleus, hypersignal in the insular cortex and adjacent white matter. CT presented with hypoattenuation of the left frontal lobe and parieto-occipital white matter associated with atrophy of the head of the caudate nucleus of the same side and enlargement of the adjacent fissures and sulcus. A subsequent PET-CT showed a left cerebral hemisphere hypometabolism, predominantly in the basal ganglia, temporal, frontal and insular lobes. Functional evaluation detected diffuse unilateral disorganization of brain electric activity, but no epileptic paroxysmal discharges were found on the electroencephalogram. After hospitalization, human immunoglobulin at the dose 2g/kg/day was used for five days. The girl remained stable, with no complications. After sixteen days, she was discharged with a rehabilitation program and follow-up care

Consultas neurológicas e diagnósticos em um grande hospital universitário dedicado a COVID-19

Background: More than one-third of COVID-19 patients present neurological symptomsranging from anosmia to stroke and encephalopathy. Furthermore, pre-existingneurological conditions may require special treatment and may be associated with worseoutcomes. Notwithstanding, the role of neurologists in COVID-19 is probablyunderrecognized. Objective: The aim of this study was to report the reasons forrequesting neurological consultations by internists and intensivists in a COVID-19-dedicated hospital. Methods: This retrospective study was carried out at Hospital dasClínicas da Faculdade de Medicina da Universidade de São Paulo, Brazil, a 900-bedCOVID-19 dedicated center (including 300 intensive care unit beds). COVID-19 diagnosiswas confirmed by SARS-CoV-2-RT-PCR in nasal swabs. All inpatient neurologyconsultations between March 23rd and May 23rd, 2020 were analyzed. Neurologistsperformed the neurological exam, assessed all available data to diagnose theneurological condition, and requested additional tests deemed necessary. Difficultdiagnoses were established in consensus meetings. After diagnosis, neurologists wereinvolved in the treatment. Results: Neurological consultations were requested for 89 outof 1,208 (7.4%) inpatient COVID admissions during that period. Main neurologicaldiagnoses included: encephalopathy (44.4%), stroke (16.7%), previous neurologicaldiseases (9.0%), seizures (9.0%), neuromuscular disorders (5.6%), other acute brainlesions (3.4%), and other mild nonspecific symptoms (11.2%). Conclusions: Mostneurological consultations in a COVID-19-dedicated hospital were requested for severeconditions that could have an impact on the outcome. First-line doctors should be able torecognize neurological symptoms; neurologists are important members of the medicalteam in COVID-19 hospital care.Introdução: Mais de um terço dos pacientes com COVID-19 apresentam sintomasneurológicos que variam de anosmia a AVC e encefalopatia. Além disso, doençasneurológicas prévias podem exigir tratamento especial e estar associadas a pioresdesfechos. Não obstante, o papel dos neurologistas na COVID-19 é provavelmentepouco reconhecido. Objetivo: O objetivo deste estudo foi relatar os motivos para solicitarconsultas neurológicas por clínicos e intensivistas em um hospital dedicado à COVID-19. Métodos: Estudo retrospectivo realizado no Hospital das Clínicas da Faculdade deMedicina da Universidade de São Paulo, Brasil, um centro dedicado à COVID-19 com900 leitos (incluindo 300 leitos para unidades de terapia intensiva). O diagnóstico deCOVID-19 foi confirmado por SARS-CoV-2-RT-PCR em swabs nasais. Todas asinterconsultas de neurologia hospitalar entre 23 de março e 23 de maio de 2020 foramanalisadas. Os neurologistas realizaram o exame neurológico, avaliaram todos os dadosdisponíveis para diagnosticar a patologia neurológica e solicitaram exames adicionaisconforme necessidade. Diagnósticos difíceis foram estabelecidos em reuniões deconsenso. Após o diagnóstico, os neurologistas participaram da condução dos casos.Resultados: Foram solicitadas consultas neurológicas para 89 de 1.208 (7,4%) empacientes internados por COVID-19 durante o período. Os principais diagnósticosneurológicos incluíram: encefalopatia (44,4%), acidente vascular cerebral (16,7%),doenças neurológicas prévias (9,0%), crises epilépticas (9,0%), transtornosneuromusculares (5,6%), outras lesões encefálicas agudas (3,4%) e outros sintomasleves inespecíficos (11,2%). Conclusões: A maioria das consultas neurológicas em umhospital dedicado à COVID-19 foi solicitada para condições graves que poderiam afetaro desfecho clínico. Os médicos na linha de frente devem ser capazes de reconhecersintomas neurológicos. Os neurologistas são membros importantes da equipe médica noatendimento hospitalar à COVID-19

Gerstmann's syndrome and unilateral optic ataxia in the emergency department

ABSTRACT. A 75-year-old right-handed woman presented to the emergency department with simultanagnosia and right unilateral optic ataxia. Moreover, the patient had agraphia, acalculia, digital agnosia and right-left disorientation, consistent with complete Gerstmann's syndrome. This case highlights the concurrence of Gerstmann's syndrome and unilateral optic ataxia in the acute phase of a left middle cerebral artery stroke

Gerstmann's syndrome and unilateral optic ataxia in the emergency department

<div><p>ABSTRACT. A 75-year-old right-handed woman presented to the emergency department with simultanagnosia and right unilateral optic ataxia. Moreover, the patient had agraphia, acalculia, digital agnosia and right-left disorientation, consistent with complete Gerstmann's syndrome. This case highlights the concurrence of Gerstmann's syndrome and unilateral optic ataxia in the acute phase of a left middle cerebral artery stroke.</p></div