Neurological implications of SARS-CoV-2 infection: review of literature

The infection caused by the new coronavirus had its first case described in December 2019, in Wuhan, China, and reached a pandemic status in March 2020. Since then, knowledge about the different aspects of this infection has evolved, as well as increased reports concerning related neurological manifestations. Thus, the neurologist assumes a fundamental role in the care of these patients, who may have a clinical phenotype that goes beyond respiratory aspects. In the present study, we highlight the data available in the literature so far regarding the main neurological implications related to COVID-19 infection, in addition to calling attention for some aspects related to patients with previous neurological diseases who contract this infection

Fabry’s Disease and Dysferlinopathy: co-occurence of two rare genetic disorders

Fabry’s Disease (FD) is a rare X-linked inherited disorder of glycosphingolipid metabolism due to absent or deficient activity of α-galactosidase A (GLA) enzyme that can present with multisystemic involvement, including painful small fiber neuropathy. Dysferlinopathy is a heterogeneous spectrum of neuromuscular disorders inherited in an autosomal recessive manner. In this report, we present a case of co-occurrence of these two rare genetic disorder

Diabetic Amyotrophy co-occurring with treatment induced neuropathy of diabetes (TIND): a case report

Diabetic amyotrophy is a rare condition in diabetic patients, usually associated with important weight loss. In the present case, we show a rare presentation of diabetic amyotrophy phenotype in the context of treatment induced neuropathy of diabetes neuropathy (TIND) after a strict glycemic control in a patient with long-standing poor controlled diabetes and without related weight loss. Even though it’s an uncommon presentation, because of diabetes high prevalence worldwide the knowledge of its complications is important to the adequate treatment and follow-up to improve patients’ quality of life.&nbsp

Perfil clínico, neurofisiológico e histopatológico de pacientes com hanseníase e dor neuropática em centros de referência neuromuscular no Estado do Rio de Janeiro

A hanseníase é doença infecciosa granulomatosa crônica que figura como problema de saúde pública no Brasil. Essa infecção é uma causa comum de neuropatia periférica em nosso meio, manifestando-se com dor neuropática (DN) em uma considerável parcela dos casos. Os fatores de risco para a ocorrência de dor neuropática nos pacientes com hanseníase ainda são pouco estabelecidos, bem como as características clínicas e de exames complementares que definem esses pacientes. Avaliamos neste estudo os aspectos clínicos, neurofisiológicos e histopatológicos de 18 pacientes com neuropatia hansênica e dor neuropática. Dentre os pacientes estudados, procuramos correlacionar as variáveis clínicas, de eletroneuromiografia (ENMG), dados morfológicos da biópsia de nervo e indicadores de qualidade de vida. Dentre os dados analisados, encontramos correlação estatisticamente significativa (p-valor < 0,05) entre a duração da hanseníase e a duração da dor neuropática associada, e também entre a idade do paciente e o número de nervos afetados na ENMG. Também encontramos relação inversa, estatisticamente significativa (p-valor < 0,05), entre o tempo de dor neuropática e a pontuação no domínio “estado geral da saúde” no questionário de qualidade de vida SF-36, marcando um pior indicador de qualidade de vida entre os pacientes com DN mais prolongada. Identificamos a presença de infiltrado inflamatório na biópsia de nervo da maioria dos pacientes com reação hansênica, e também pior pontuação no domínio “dor" do SF-36 entre esses pacientes; esses dados em conjunto reforçam a premissa conhecida de que a presença de reação hansênica seja um fator de risco para a ocorrência de dor neuropática. Corroboramos, portanto, os dados já estabelecidos de que a dor neuropática é achado bem estabelecido entre os pacientes com neuropatia associada à hanseníase. Concluímos que o conhecimento sobre os fatores de risco e as características associadas à DN nesses pacientes é de potencial importância para traçarmos estratégias que contemplem identificação precoce e tratamento dessa manifestação associada à hanseníase. Além disso, demonstramos que a DN associada à hanseníase impacta negativamente nos indicadores de qualidade de vida dos pacientes, o que reforça ainda mais a importância desse tema na prática neurológicaLeprosy is a chronic granulomatous infectious disease that appears as a public health problem in Brazil. This infection is a common cause of peripheral neuropathy, presenting with neuropathic pain (NP) in a considerable number of cases. The risk factors for the occurrence of neuropathic pain in leprosy patients are still poorly established, as well as the clinical and complementary tests characteristics that define these patients. In this study, we evaluated the clinical, neurophysiological and histopathological aspects of 18 patients with leprosy neuropathy and neuropathic pain. Among the patients studied, we sought to correlate the clinical variables, electroneuromyography (ENMG), morphological data of nerve biopsy and quality of life indicators. Among the data analyzed, we found a statistically significant correlation (p-value <0.05) between the duration of leprosy and the duration of associated neuropathic pain, and also between the age of the patient and the number of nerves affected in ENMG. We also found an inverse relationship, statistically significant (pvalue <0.05), between the duration of neuropathic pain and the score in the domain “general health” in SF-36, marking a worse indicator of quality of life among patients with longer NP. We identified the presence of inflammatory infiltrate in the nerve biopsy of most patients with leprosy reaction, and also worse scores in the SF-36 “pain” domain among these patients; these data together reinforce the known premise that the presence of leprosy reaction is a risk factor for the occurrence of neuropathic pain. We therefore corroborate the already established data that neuropathic pain is a well-established finding among patients with neuropathy associated with leprosy. We conclude that knowledge about risk factors and characteristics associated with NP in these patients is of potential importance to outline strategies that contemplate early identification and treatment of this manifestation associated with leprosy, in addition to demonstrating that NP associated with leprosy negatively impacts the quality of life indicators of patients, which further reinforces the importance of this topic in neurologic practice.110 f

Multiple cranial nerve palsies in malignant external otitis: A rare presentation of a rare condition

Malignant external otitis (MEO) is a rare inflammatory and infectious condition, typically caused by Pseudomonas aeruginosa, that mainly affects diabetic or immunocompromised elderly patients and is associated with severe morbidity and mortality. It begins in the external auditory canal and rapidly progresses through the skull base, leading to osteomyelitis and may result in cranial neuropathy, especially of the facial nerve. Here we describe a rare neurological presentation of MEO in a 65-year old diabetic man, who presented with an 8-month progressing left otitis externa and evolved with ipsilateral proptosis, ophthalmoplegia, blindness, facial palsy, hearing loss and contralateral evolvement of the temporal bone with hearing impairment. He was initially treated with oral ciprofloxacin and after one week was transferred to our tertiary hospital, where antibiotic therapy was switched to meropenem and vancomycin due to the severity of the case and to the hospital’s microbiological profile. The patient underwent left canal wall-up mastoidectomy with insertion of ear ventilation tube bilaterally, with good recovery of right ear hearing capacity, but with no improvements of neurological deficits nor left hearing function. All microbiological tests performed were negative, and this was interpreted as a possible consequence of the early use of antibiotics. Unfortunately, the patient was infected by Sars-CoV-2 during hospitalization and passed away after ten days of COVID-19 intensive care unit internment