Characteristics and management of Susac syndrome in an emergent country: a multi-center case series from Brazil.

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 Brazil and discuss management challenges in emergent countries. METHODS: This is a retrospective case series of patients diagnosed with SS in three medical centers in Brazil between April 2018 and July 2021. The European Susac consortium (EuSaC) criteria were used for diagnosis of SS. Demographic data and clinical interventions were described and outcomes were assessed subjectively and by applying the modified Rankin Scale (mRS) on last follow-up. RESULTS: Six patients were diagnosed with SS (3 males, 3 females). Mean age at presentation was 36 years (range 17 to 54). The most common initial symptom was confusion, followed by visual impairment and hearing loss. Characteristic snowball lesions on magnetic resonance imaging (MRI) were present in four patients (66%). Retinal artery abnormalities were present in half (3/6) of patients, and sensorineural hearing loss was present in four patients (66%). Outcome was favorable (mRS ≤ 2) in five patients (86%). Patients treated early had a more favorable outcome. CONCLUSION: Emergent countries face challenges in the diagnosis and management of patients with SS, such as access to advanced tests (fluorescein angiography, serial MRI) and treatment drugs (rituximab, mycophenolate). Further research should consider particularities of patients with SS in emergent countries.

Normal pressure hydrocephalus associated with COVID-19 infection: a case report.

BACKGROUND: COVID-19 is a pandemic disease responsible for many deaths worldwide. Many neurological manifestations have been described. We report a case of normal pressure hydrocephalus (NPH) 2 months after acute COVID19 infection, in a patient without other risk factors. CASE PRESENTATION: A 45-year-old male patient presented an 8-month history of progressive gait disorder and cognitive impairment after being hospitalized for SARS-CoV-2 infection. Magnetic resonance imaging (MRI) was compatible with NPH. A spinal tap test was positive and there was progressive improvement after shunting, with complete resolution of symptoms. CONCLUSION: Other infections such as syphilis, cryptococcosis and Lyme disease have been associated with NPH. Possible mechanisms for NPH after COVID include disruption of choroid plexus cells by direct viral invasion or as a result of neuroinflammation and cytokine release and hypercoagulability leading to venous congestion and abnormalities of CSF flow. Given the significance of NPH as a cause of reversible dementia, it is important to consider the possibility of a causal association with COVID19 and understand the mechanisms behind this association.

Application of 68Ga-PSMA-11 PET/CT in the Diagnosis of Prostate Cancer Clinical Relapse.

BACKGROUND: This work aims to present a nuclear medicine imaging service's data regarding applying positron emission-computing tomography (PET/CT) scans with the radiopharmaceutical 68Ga-PSMA-HBED-CC (68Ga-PSMA-11) to diagnose prostate cancer clinical relapse. METHODS: Eighty patients with a mean age of 68.26 years and an average prostatic-specific antigen blood level of 7.49 ng/ml (lower concentration = 0.17 ng/ml) received 68Ga-PSMA-11 intravenously, and full-body images of PET-CT scan were obtained. Of the total of patients admitted to the imaging service, 87.5% were examined for disease's biochemical recurrence and clinical relapse, and 70.0% had a previous radical prostatectomy (RP). RESULTS: Of the patients without RP, 95.8% were detected with intra-glandular disease. The 68Ga- PSMA-11 PET/CT imaging results revealed small lesions, even in patients with low blood levels of prostatic-specific antigen, mainly in metastatic cancer cases in lymph nodes and bones. CONCLUSION: The 68Ga-PSMA-11 PET/CT imaging was essential in detecting prostate cancer, with significantly high sensitivity in detecting recurrent cases. Due to its inherent reliability and sensitivity, PET/CT scanning with 68Ga-PSMA-11 received an increasing number of medical requests throughout the present follow-up study, confirming the augmented demand for this clinical imaging procedure in the regional medical community.

Assessment of Sensitivity and Specificity of Ultrasound and Magnetic Resonance Imaging in the Diagnosis of Placenta Accreta.

OBJECTIVE: To assess and compare the sensitivity and specificity of ultrasonography and magnetic resonance imaging in the diagnosis of placenta accreta in patients with placenta previa. METHODS: This retrospective cohort study included 37 women, and was conducted between January 2013 and October 2015; 16 out of the 37 women suffered from placenta accreta. Histopathology was considered the gold standard for the diagnosis of placenta accreta; in its absence, a description of the intraoperative findings was used. The associations among the variables were investigated using the Pearson chi-squared test and the Mann-Whitney U-test. RESULTS: The mean age of the patients was 31.8 ± 7.3 years, the mean number of pregnancies was 2.8 ± 1.1, the mean number of births was 1.4 ± 0.7, and the mean number of previous cesarean sections was 1.2 ± 0.8. Patients with placenta accreta had a higher frequency of history of cesarean section than those without it (63.6% versus 36.4% respectively; p < 0.001). The mean gestational age at birth among women diagnosed with placenta previa accreta was 35.4 ± 1.1 weeks. The mean birth weight was 2,635.9 ± 374.1 g. The sensitivity of the ultrasound was 87.5%, with a positive predictive value (PPV) of 65.1%, and a negative predictive value (NPV) of 75.0%. The sensitivity of the magnetic resonance imaging was 92.9%, with a PPV of 76.5%, and a NPV of 75.0%. The kappa coefficient of agreement between the 2 tests was 0.69 (95% confidence interval [95%CI]: (0.26-1.00). CONCLUSION: The ultrasound and the magnetic resonance imaging showed similar sensitivity and specificity for the diagnosis of placenta accreta.

OBJETIVO: Avaliar e comparar a sensibilidade e especificidade da ultrassonografia e da ressonância magnética no diagnóstico do acretismo placentário em pacientes com placenta prévia. MéTODOS: Estudo de coorte retrospectivo com 37 mulheres, sendo 16 com acretismo placentário, realizado de janeiro de 2013 a outubro de 2015. Considerou-se padrão-ouro para o diagnóstico de acretismo placentário o exame anatomopatológico, sendo que, na sua ausência, a descrição do achado intraoperatório. As associações entre variáveis foram investigadas utilizando o teste qui-quadrado de Pearson e o teste U de Mann-Whitney. RESULTADOS: A idade média foi de 31,8 ± 7,3 anos, o número médio de gestações foi de 2,8 ± 1,1, o número médio da quantidade de partos foi de 1,4 ± 0,7, e o número médio de cesáreas prévias foi de 1,2 ± 0,8. O grupo do acretismo placentário apresentou antecedente de cesariana mais frequentemente do que o grupo sem acretismo (63,6% versus 36,4%, respectivamente; p < 0,001). A idade gestacional no parto em mulheres com diagnóstico de placenta prévia com acretismo foi de 35,4 ± 1,1 semanas. O peso ao nascer médio foi de 2.635,9 ± 374,1 g. A sensibilidade do ultrassom foi de 87,5%, com valor preditivo positivo (VPP) de 65,1%, e valor preditivo negativo (VPN) de 75,0%. Para a ressonância magnética, a sensibilidade foi de 92,9%, com VPP de 76,5% e VPN de 75,0%. O índice kappa para concordância entre os dois testes foi de 0,69 (intervalo de confiança de 95% [IC95%]: 0,26­1,00). CONCLUSãO: O ultrassom e a ressonância magnética apresentaram sensibilidade e especificidade semelhantes no diagnóstico do acretismo placentário.

Magnetic resonance imaging of sellar and juxtasellar abnormalities: atypical findings of common diseases and typical findings of rare diseases.

The sellar/juxtasellar region comprises the bone component of the sella turcica, pituitary gland, cavernous sinus, and suprasellar cistern. Abnormalities in this region can be attributed to underproduction or overproduction of hormones or to the neurological signs and symptoms resulting from the compression of adjacent structures. Magnetic resonance imaging (MRI) is currently the imaging method of choice, having supplanted computed tomography. The aim of this study was to demonstrate the common and uncommon imaging aspects of sellar and juxtasellar changes, which could facilitate the differential diagnosis. We retrospectively evaluated the MRI scans of 70 patients with sellar/juxtasellar abnormalities from didactic files, and report those with more unusual changes, where MRI played an important role in diagnosis. All cases were confirmed histologically or clinical laboratory.

Pyle disease (metaphyseal dysplasia) presenting in two adult sisters.

Pyle's disease is an extremely rare skeletal disorder characterized by a benign course and an autosomal recessive genetic pattern of inheritance. Its causal mutation is still unknown. In the medical literature, fewer than 30 cases have been described to date. We report the case of two female siblings, daughters of consanguineous parents, referred to the radiology department complaining of genu valgum. Laboratory tests showed no other relevant findings. Conventional radiography plain films revealed Erlenmeyer flask deformity in bilateral femorotibial metaphyses, metaphyseal flaring of long bones, and mild sclerosis of the skull base. The clinicoradiological dissociation, along with the characteristic imaging findings, was consistent with the diagnosis of Pyle's disease. Intervention is not required in most cases, but orthopedic treatment may be required for genu valgum or fractures. Therefore, these cases emphasize the pivotal role conventional radiography plays in the correct diagnosis of this rare entity, allowing for appropriate genetic counseling.

Vascular cognitive impairment in Pemphigus vulgaris: A case report.

Pemphigus vulgaris is a systemic auto-immune medical condition that mainly manifests with changes in skin and vasculopathy. This is a case report of a 69-year-old male with confirmed histopathologic diagnosis of Pemphigus vulgaris presenting ulterior Cognitive Impairment, mostly in executive function. The patient was treated using steroids, immunomodulatory therapy, fluoxetine and galantamine. Neuropsychological testing and magnetic resonance (MRI) were performed. This is the first report of correlational cognitive impairment with Pemphigus vulgaris in the literature. Physicians should be aware of vascular causes for cognitive impairment in patients presenting auto-immune conditions.

Phemphigus vulgaris é uma condição médica sistêmica autoimune que principalmente se manifesta com alterações de pele e vasculopatia. Este é um caso de um homem de 69 anos com diagnóstico histopatológico de Phemphigus vulgaris apresentando posterior comprometimento cognitivo, predominante em funções executivas. O paciente foi tratado com esteroides, terapia imunomoduladora, fluoxetina e galantamina. Avaliação neuropsicológica e ressonância magnética foram realizados. Este é o primeiro relato correlacionando comprometimento cognitivo a pênfigo vulgar na literatura. Os clínicos devem estar cientes das causas vasculares para comprometimento cognitivo em pacientes se apresentando com condições autoimunes.

[Isolated central nervous system angiitis and myelodysplastic syndrome: case report]. / Angeíte isolada do sistema nervoso central em paciente com síndrome mielodisplásica: relato de caso.

Isolated central nervous system (CNS) angiitis are vasculitides of undetermined etiology in which only nervous system vessels are affected. In most cases there is no associated systemic disease. We report the case of a 67 years old man with previous hematologic diagnosis of myelodysplastic syndrome who developed an isolated CNS angiitis.