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.

Assessing small fiber neuropathy and subtle cardiac involvement in Fabry disease

Abstract Fabry disease (FD) is an X-linked lysosomal storage disorder characterized by reduced or absent activity of the enzyme α-galactosidase A. Due to systemic accumulation of glycolipids, FD phenotype is diverse, and diagnosis may be challenging. Clinical manifestations include small fiber neuropathy, renal dysfunction, cardiac involvement, cerebrovascular disease, among others. In the present study, we describe biopsy proven small fiber neuropathy and subclinical cardiac involvement in two cousins diagnosed with FD secondary to a recently described pathogenic variant, highlighting the importance of diagnostic tools to document organ damage and allow early treatment.

Neurological implications of SARS-CoV-2 infection: review of literature / Implicações neurológicas da infecção por SARS-CoV-2: revisão da literatura

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.

A infecção causada pelo novo Coronavírus teve seu primeiro caso descrito em dezembro de 2019, em Wuhan, China e alcançou o status de pandemia em março de 2020. Desde então, o conhecimento sobre os diferentes aspectos da referida infecção evolui assim como aumentam relatos de manifestações neurológicas relacionadas. Assim, o neurologista assume papel fundamental na assistência desses pacientes, que podem ter um fenótipo clínico que ultrapassa os aspectos respiratórios. No presente estudo, destacamos os dados disponíveis na literatura até o presente momento no tocante às principais implicações neurológicas relacionadas à infecção pelo COVID-19, além de destacar alguns aspectos relativos aos pacientes com doenças neurológicas prévias que contraem a referida infecção.