Guilherme Diogo Silva

University of São Paulo | USP · Department of Neurology

A história das doenças autoinflamatórias remonta a séculos, com as primeiras observações e descrições encontradas em textos médicos antigos e registos históricos. Embora as descrições pormenorizadas das síndromes de febre periódica familiar só tenham surgido na segunda metade do século XX, as febres periódicas têm sido documentadas desde a antiguid...

La historia de las enfermedades autoinflamatorias se remonta a siglos atrás, con observaciones y descripciones tempranas encontradas en textos médicos antiguos y registros históricos. Aunque las descripciones detalladas de los síndromes de fiebre periódica familiar no aparecieron hasta la segunda mitad del siglo XX, las fiebres periódicas se han do...

The history of autoinflammatory diseases goes back centuries, with early observations and descriptions found in ancient medical texts and historical records. Although detailed descriptions of familial periodic fever syndromes only appeared in the second half of the 20th century , periodic fevers have been documented since antiquity. Since the first...

Background Monogenic autoinflammatory disorders result in a diverse range of neurological symptoms in adults, often leading to diagnostic delays. Despite the significance of early detection for effective treatment, the neurological manifestations of these disorders remain inadequately recognized. Methods We conducted a systematic review searching...

Background The clinical features of central nervous system (CNS) sporotrichosis are derived from case reports and a limited series of cases. Our objective was to carry out a systematic review and meta‐analysis of CNS sporotrichosis. Methods We searched PubMed/MEDLINE, Embase, Scopus, and LILACS on 9 September 2023. Our inclusion criteria were docu...

Swallowing is a complex function that can be disrupted after stroke. Transcranial Direct Current Stimulation (tDCS) is a non-invasive brain stimulation therapy that recently has been tested to treat stroke-related dysphagia. The authors performed a search in the literature to review the described evidence of the use of tDCS in dysphagia after strok...

Background Multiple sclerosis misdiagnosis remains a problem despite the well-validated McDonald 2017. For proper evaluation of errors in the diagnostic process that lead to misdiagnosis, it is adequate to incorporate patients who are already under regular follow-up at reference centers of demyelinating diseases. Objectives To evaluate multiple sc...

Background: To report a case of IgG4-related pachymeningitis presenting with cystic lesions mimicking neurocysticercosis. Case presentation: A 40-year-old female patient with tetraparesis, dysphagia and dysphonia was evaluated with clinical examination, magnetic resonance imaging, and meningeal biopsy. Magnetic resonance imaging (MRI) revealed dif...

Anti-NMDA receptor encephalitis (NMDARE), an autoimmune encephalitis associated with autoantibodies against the N-methyl-D-aspartate (NMDA) receptor, affects predominantly young women and is associated with psychiatric symptoms , seizures, movement disorders, and autonomic instability. Traditional treatments of anti-NMDA receptor encephalitis invol...

Supratentorial Lymphocytic Inflammation with Parenchymal Perivascular Enhancement Responsive to Steroids (SLIPPERS) is a rare variant of the CLIPPERS spectrum with less than ten reports published so far. There is ongoing discussion regarding whether SLIPPERS is a disease entity on its own or just an acronym encompassing many underlying diagnoses, s...

Background A clear cutoff value of galactomannan (GM) has not been established for chronic pulmonary aspergillosis (CPA) and is frequently extrapolated from invasive pulmonary aspergillosis. We performed a systematic review and meta-analysis to evaluate the diagnostic performance of serum and bronchoalveolar lavage (BAL) GM, and to propose a cutoff...

Optic neuritis (ON) admits diverse differential diagnoses. Petzold proposed diagnostic criteria for ON in 2022, although real-world application of these criteria is missing. We conducted a retrospective review of patients with ON. We classified patients into definite or possible ON, and into groups A (typical neuritis), B (painless), or C (binocula...

Introduction The deficiency of ADA2 (DADA2) is a rare autoinflammatory disease provoked by mutations in the ADA2 gene inherited in a recessive fashion. Up to this moment there is no consensus for the treatment of DADA2 and anti-TNF is the therapy of choice for chronic management whereas bone marrow transplantation is considered for refractory or se...

Background: The knowledge of central nervous system (CNS) histoplasmosis is limited to case reports and series. Objectives: Our objective was to synthesise clinical, radiological and laboratory characteristics of CNS histoplasmosis to improve our understanding of this rare disease. Methods: We performed a systematic review using Pubmed/MEDLINE...

Background: Vaccination in patients with neuromyelitis optica spectrum disorders (NMOSD) is challenging because there is a concern that vaccines can lead to clinical attacks. However, little is known about the risk and the characteristics of attacks occurring after vaccination. Methods: We performed a systematic review and meta-analysis using Pu...

Introduction: The de ciency of ADA2 (DADA2) is a rare autoin ammatory disease provoked by mutations in the ADA2 gene inherited in a recessive fashion. Up to this moment there is no consensus for the treatment of DADA2 and anti-TNF is the therapy of choice for chronic

Introduction: The deficiency of ADA2 (DADA2) is a rare autoinflammatory disease provoked by mutations in the ADA2 gene inherited in a recessive fashion. Up to this moment there is no consensus for the treatment of DADA2 and anti-TNF is the therapy of choice for chronic management whereas bone marrow transplantation is considered for refractory or s...

Background: Optic neuritis (ON), a major cause of visual impairment in young adults, is generally associated with rapid visual recovery when treated with intravenous methylprednisolone treatment (IVMPT). However, the optimal duration of such treatment is unknown, ranging from three to seven days in clinical practice. We aimed to compare the visual...

Retinal complications in patients with inflammatory optic neuritis (ON) are generally related to post-infectious neuroretinitis and are considered uncommon in autoimmune/demyelinating ON, whether isolated or caused by multiple sclerosis (MS) or neuromyelitis optica spectrum disorder (NMOSD). More recently, however, cases with retinal complications...

Background: The optimal treatment strategy of multiple sclerosis (MS) is a matter of debate. The classical approach is the escalating (ESC) strategy, which consists of starting with low- to moderate-efficacy disease-modifying drugs (DMDs) and upscale to high-efficacy DMDs when noting some evidence of active disease. Another approach, the early int...

Background Chronic meningitis (CM) is characterized by neurological symptoms associated with the evidence of cerebrospinal fluid pleocytosis lasting > 4 weeks. Studies on the management of CM in Brazil are scarce. Objective To critically review the literature on CM and propose a rational approach in the Brazilian scenario. Methods Narrative literat...

Background: Numerous studies addressed the prevalence of multiple sclerosis, but prevalence studies of NMOSD and, particularly, MOGAD are scarce. We aimed to estimate the prevalence of NMOSD and MOGAD in the city of São Paulo, based on the known prevalence of MS. Methods: In this observational study, we determined the total number of patients wi...

Background: There is concern that recommendations on prophylactic antiseizure drugs (PASDs) for patients with spontaneous intracerebral hemorrhage (sICH) are biased by studies using older drugs and no electrographic monitoring. Aims: We performed a systematic review and meta-analysis to determine whether PASDs in patients with sICH reduced seizure...

Current understanding of anti-Yo/PCA1 antibody-associated cerebellar ataxia is based on case reports and small case series. Our goal was to summarize clinical features, highlighting atypical presentations and gaps of knowledge. Following the PRISMA guidelines, we systematically screened Pubmed/MEDLINE, Embase, Scopus, and Web of Science from incept...

Background Multiple sclerosis (MS) and neuromyelitis optica spectrum disorders (NMOSD) are the most common autoimmune diseases of the central nervous system (CNS). They present chronic relapsing courses that demand treatment with disease-modifying drugs (DMDs) to prevent inflammatory activity. Disease-modifying drugs lead to immunomodulation or imm...

Background Primary angiitis of the central nervous system is a rare inflammatory vasculopathy and it is a difficult diagnosis to make because of its kaleidoscopic presentation and its multiple mimics, including multiple sclerosis. Case presentation A 21-year-old men presented a four-year history of progressive gait deterioration. Magnetic resonanc...

Ocrelizumab and siponimod are the two on‐label drugs used for progressive forms of multiple sclerosis (PMS). However, many patients with PMS do not have access to these high‐efficacy disease‐modifying drugs (DMDs). Off‐label prescription of other high‐efficacy DMDs (fingolimod, rituximab and natalizumab) may be a strategy to improve access to immun...

Background: Advanced neuroimaging demonstrated that neurological involvement occurs in up to 30% of paracoccidioidomycosis (PCM) cases. Current knowledge of neuroparacoccidioidomycosis (NPCM) is based on a 2009 systematic review. However, in the last decade several new cases have been published, with modern neuroimaging techniques. Objectives: W...

Magnetic stimulation is a safe, non-invasive diagnostic tool and promising treatment strategy for neurological and psychiatric disorders. Although most studies address transcranial magnetic stimulation, transspinal magnetic stimulation (TsMS) has received recent attention since trials involving invasive spinal cord stimulation showed encouraging re...

Background: Susac syndrome (SS) is a rare endotheliopathy with an estimated prevalence of 0.14-0.024 per 100,000. It is an important differential diagnosis in demyelinating disorders. There are few case series and no large randomized controlled trials, and most reports come from developed countries. We report six cases of SS in three centers in Br...

Autoimmune encephalitis (AE) comprises a group of diseases mediated by antibodies against neuronal cell surface or synaptic antigens, such as ion channels or neurotransmitter receptors. New clinical syndromes and their associated antibodies were and are still being characterized over the last two decades. The fact that their main clinical features...

Objective: To obtain feedback from Neurology residents and Neuroimmunology fellows about the practical training in multiple sclerosis (MS). Methods: A survey was developed and administered electronically to Neurology residents or Neuroimmunology fellows that received training in eight large MS outpatient clinics in Brazil, from 2018 to 2021. We...

A neuroesquistossomose tipicamente apresenta quadro de mielorradiculite. Porém, relatamos a seguir uma encefalite de tronco (romboencefalite). Mulher, 25 anos, sem comorbidades prévias, procedente de São Paulo, admitida com quadro progressivo de tontura, cefaleia, parestesia à direita, evoluindo com diplopia e ataxia de marcha há uma semana. Relato...

The relationship between severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) and neurodegenerative diseases is yet to be fully clarified. Rapid worsening and even new-onset cases of those disorders have been reported in association with coronavirus disease 2019 (COVID-19). We describe three cases of neurodegenerative diseases in patients w...

Introduction: Acute flaccid paralysis (AFP) is characterized by progressive weakness with signs of impairment of the lower motoneuron. Secondary hyperkalemic paralysis is a cause of AFP that must be quickly recognized to prevent cardiac and neurological deterioration. Methods: We present a case of secondary hyperkalemic paralysis admitted to the em...

Background The typical brainstem syndrome associated with aquaporin-4-positive neuromyelitis optica spectrum disorder (AQP4-positive NMOSD) is the area postrema syndrome, whereas brainstem neuro-ophthalmological syndromes, such as internuclear ophthalmoplegia, are typical of multiple sclerosis. Half-and-half syndrome is a rare brainstem syndrome de...

Guillain-Barre syndrome following COVID-19 vaccines (GBSfCV19v) is a reported adverse effect that remains unclear. We present a structured review based on two case reports of GBSfCV19v, a systematic review, and Vaccine Adverse Event Reporting System (VAERS) analysis to estimate the risk and describe the clinical characteristics (CC) of these events...

Objectives Our goal was to describe outcomes in a single-center, real-world series of patients with acute basilar artery occlusion in a middle-income country. In addition, we assessed potential outcome predictors. Material and Methods Data from 28 patients were retrospectively reviewed. The primary outcome was death until last follow-up. Other out...

The main objective of this article is to improve our understanding of the differences and similarities of these two anti-gamma-aminobutyric acid receptor encephalitis, anti-GABAaR and anti-GABAbR. The data were systematically collected and we found 26 studies: seven studies and 37 patients corresponded to anti-GABAaR encephalitis, and 21 manuscript...

Background For the last 20 years, the amyloid‐beta (Aβ) cascade hypothesis has been dominant in the research of disease‐modifying therapeutics, with spent of financial and clinical resources in potential drugs targeting Aβ peptide production or clearance. (1) In this context, monoclonal antibodies targeting beta‐amyloid (Aβ‐mAbs) are being tested t...

Rapidly progressive cerebellar ataxia is a classical paraneoplastic neurological syndrome associated with different autoantibodies and typical demographic characteristics, extracerebellar signs, tumor association, and prognosis. Anti-Tr/anti-Delta/Notch-like epidermal growth factor-related receptor (DNER) antibody is one of the associated antibodie...

Objective To present oral 1g methylprednisolone (O1M) as treatment for mild relapses in patients with neuromyelitis optica (pwNMO). Background O1M is a cost-reducing non-inferior treatment for relapses in patients with multiple sclerosis but little is known about this strategy in pwNMO. Design/methods Two case reports to highlight the feasibility...

The Scientific Department of Neuroimmunology of the Brazilian Academy of Neurology (DCNI/ABN) and Brazilian Committee for Treatment and Research in Multiple Sclerosis and Neuroimmunological Diseases (BCTRIMS) provide recommendations in this document for vaccination of the population with demyelinating diseases of the central nervous system (CNS) ag...

Introduction: Although neurological complications are well recognized in patients with sickle cell disease, myelopathy has been rarely described, with few reported cases of compressive and ischemic myelopathy. We present the first case report of longitudinally extensive myelitis (LETM) in SCD and review the differential diagnosis of myelopathy in...

Observational Study Neurol Neuroimmunol Neuroinflamm . 2021 Aug 26;8(6):e1060. doi: 10.1212/NXI.0000000000001060. Print 2021 Nov. Clinical Features of COVID-19 on Patients With Neuromyelitis Optica Spectrum Disorders Samira Luisa Apostolos-Pereira 1, Lis Campos Ferreira 1, Mateus Boaventura 1, Nise Alessandra de Carvalho Sousa 1, Gabriela Joca Mart...

Background: Chorea is a movement disorder characterized by random, brief and migratory involuntary muscle contractions. It is defined as acute when present within hours to days. Three main causes for this scenario have emerged as most likely: vascular, toxic-metabolic and inflammatory. Objectives: To identify the prevalence of the main etiologies...

Context: Acute flaccid paralysis (AFP) is characterized by progressive weakness with signs of impairment of the lower motoneuron. Secondary hyperkalemic paralysis is a cause of AFP that must be quickly recognized to prevent cardiac and neurological deterioration. Case report: We present a 69-years-old man admitted to the emergency department due to...

Introduction: Up to 10% of strokes affect people under 45 years old. Syphilis is most common in adults. Fourteen percent of neurosyphilis cases have stroke its first manifestation. Objectives: To discuss neurosyphilis as an overlooked etiology for stroke. Design and setting: The case occurred in a public secondary care hospital located in São Paulo...

Context: Chiropractic is a form of spinal manipulation used to treat cervical pain. This therapy is considered safer than chronic use of anti-inflammatory drugs, opioids or spine surgery. However, chiropractic may cause severe complications such as myelopathy and ischemic stroke. Case report: A 48-year-old woman was admitted to our hospital due to...

Context: Central venous catheterization of the internal jugular vein is a common procedure that can be complicated with Horner`s Syndrome, caused by a direct lesion of cervical sympathetic pathways, pneumothorax compression, or carotid dissection. This entity should be considered when assessing new anisocoria in intensive care scenarios. Case Repor...

Context: Trigeminal autonomic cephalalgias (TAC) are unilateral and recurrent headache with ipsilateral cranial autonomic symptoms such as lacrimation, conjunctival injection, ptosis and changes on pupillary diameter. Acute glaucoma is a rare etiology of headache in the emergency room and can be confused with TAC because it also presents with simil...

Context: The Man-in-the-barrel syndrome is characterized by brachial diparesis with preserved lower limb strength. This syndrome is an uncommon presentation of ischemic stroke. Cardiac surgery with or without cardiopulmonary bypass (CPB) favors hemodynamic and embolic mechanisms of stroke. Case reports: Case 1 - A 51-year-old hypertensive male pres...

Background: Although neurological complications are well recognized in sickle cell disease (SCD), myelopathy has been rarely described. We present the first case report of longitudinally extensive myelitis (LETM) in SCD and review the differential diagnosis of myelopathy in these patients. Design and setting: case-oriented review. Methods: We repor...

Context: Cardioembolic events are the main cause of simultaneous anterior and posterior circulation stroke. Anatomical variants of the circle of Willis represent another rare mecanism. Persistent trigeminal artery is a carotid–vertebrobasilar anastomosis related to an increased risk of aneurysms, vascular malformations and stroke. Case report: A 67...

Background: Studies across subspecialties of neurology (SON) report noninferiority of telemedicine (TM) compared with face-to-face intervention (FTF-I). Clinical scales (CS) are important tools for outcome measures in clinical care. However, which CS in FTF-I can be used in teleneurology is unclear. Objectives: Define the most used CS in studies co...

Approximately one third of epilepsy patients do not become seizure free with antiseizure medications. This treatment gap motivates research for new therapeutic options, such as cannabidiol (CBD). CBD differs from other cannabis derivatives because of its consistent efficacy and lack of a psychoactive effect. CBD can be recommended as adjunctive the...

Background: The novel coronavirus disease 2019 (COVID-19) pandemic poses a potential threat to patients with autoimmune disorders, including multiple sclerosis (MS) and neuromyelitis optica spectrum disorder (NMOSD). Such patients are usually treated with immunomodulatory or immunosuppressive agents, which may tamper with the organism's normal res...

Background Motor cortex stimulation (MCS) is an effective treatment in neuropathic pain refractory to pharmacological management. However, analgesia is not satisfactorily obtained in one third of patients. Given the importance of understanding the mechanisms to overcome therapeutic limitations, we addressed the question: what mechanisms can explain...