Cocaine-Induced Renal Artery Dissection as a Cause of Secondary Hypertension: A Rare Presentation.

BACKGROUND Cocaine abuse is a globally recognized problem with great socioeconomic and health impacts on society. We report a case of dissection of vertebral arteries and right renal artery after cocaine abuse that clinically presented as atypical headache and hypertension. CASE REPORT A 36-year-old male sought emergency care due to cervical pain after cocaine abuse. The pain was located to the right cervical side with irradiation to the homolateral temporal region. He had no previous comorbidities, except for cocaine abuse on a weekly basis. Angiotomography showed alterations compatible with recent arterial dissection of the right vertebral artery, confirmed on angioresonance. The patient received double anti-aggregation and antihypertensive drugs and was discharged. He was readmitted 5 days later due to hypertensive crisis and mild abdominal pain. Abdominal ultrasound with a Doppler of renal arteries showed signs right renal artery stenosis. Magnetic resonance angiography confirmed dissection of the same vessel. The patient underwent arteriography with stent implantation in the right renal artery. During outpatient follow-up, he progressed with gradual reduction of antihypertensive drugs. CONCLUSIONS There is only 1 case report correlating renal artery dissection with cocaine use and none with concomitant presentation of dissection in the vertebral and renal arterial beds. The scarcity of reports is a consequence of many problems. Therefore, young patients presenting with new-onset hypertension or abdominal pain and cocaine abuse history should raise suspicion for renal artery dissection.

Clinical and genetic basis of congenital myasthenic syndromes.

Neuromuscular junction disorders represent a wide group of neurological diseases characterized by weakness, fatigability and variable degrees of appendicular, ocular and bulbar musculature involvement. Its main group of disorders includes autoimmune conditions, such as autoimmune acquired myasthenia gravis and Lambert-Eaton syndrome. However, an important group of diseases include congenital myasthenic syndromes with a genetic and sometimes hereditary basis that resemble and mimick many of the classic myasthenia neurological manifestations, but also have different presentations, which makes them a complex clinical, therapeutic and diagnostic challenge for most clinicians. We conducted a wide review of congenital myasthenic syndromes in their clinical, genetic and therapeutic aspects.

Clinical and genetic basis of congenital myasthenic syndromes / Bases clínicas e genéticas das síndromes miastênicas congênitas

ABSTRACT Neuromuscular junction disorders represent a wide group of neurological diseases characterized by weakness, fatigability and variable degrees of appendicular, ocular and bulbar musculature involvement. Its main group of disorders includes autoimmune conditions, such as autoimmune acquired myasthenia gravis and Lambert-Eaton syndrome. However, an important group of diseases include congenital myasthenic syndromes with a genetic and sometimes hereditary basis that resemble and mimick many of the classic myasthenia neurological manifestations, but also have different presentations, which makes them a complex clinical, therapeutic and diagnostic challenge for most clinicians. We conducted a wide review of congenital myasthenic syndromes in their clinical, genetic and therapeutic aspects.

RESUMO Distúrbios da junção neuromuscular representam um grupo amplo de doenças neruológicas caracterizadas por fraqueza, fadigabilidade e graus variados de envolvimento das musculaturas apendicular, ocular e bulbar. Os principais grupos de doenças deste grupo incluem condições auto-imunes, como a miastenia gravis auto-imune adquirida e a síndrome de Lambert-Eaton. Entretanto, um outro grupo importante de doenças incluem as sindromes miastênicas congênitas com uma base genética e eventualmente hereditária que lembra e mimetiza muitas das manifestações neurológicas clássicas das miastenias, mas também se apresentam de diferentes formas tornando um desafio clínico, terapêutico e diagnóstico complexo para a maioria dos clínicos. Realizamos ampla revisão sobre as síndromes miastênicas congênitas em seus aspectos clínicos, genéticos e terapêuticos.

The challenge of managing fusiform basilar artery aneurysms: from acute ischemic stroke to a massive subarachnoid hemorrhage.

We present the case of a patient with acute brain stem ischemic stroke who was found to have a fusiform basilar aneurysm with a thrombus within the dilated vessel. Three days after the ischemic stroke, the patient had a massive subarachnoid hemorrhage and died. This case illustrates the difficulties in the acute management of ischemic events in patients with basilar fusiform aneurysms, because the natural history of this disease encompasses both bleeding and thrombosis.

Demyelinating disease in patients with myasthenia gravis.

Myasthenia gravis (MG) is an autoimmune disease characterized by fluctuating muscle weakness, caused by impaired neuromuscular transmission. Patients with MG can present other autoimmune diseases in association, commonly hypo or hyperthyroidism. The association of MG to demyelinating disease is rare and has been described before. We report on three Brazilian patients with MG that presented distinct demyelinating diseases, two monophasic and one recurrent neuromyelitis optica, several years after the diagnosis of MG, and discuss their clinical courses.

Demyelinating disease in patients with myasthenia gravis

Myasthenia gravis (MG) is an autoimmune disease characterized by fluctuating muscle weakness, caused by impaired neuromuscular transmission. Patients with MG can present other autoimmune diseases in association, commonly hypo or hyperthyroidism. The association of MG to demyelinating disease is rare and has been described before. We report on three Brazilian patients with MG that presented distinct demyelinating diseases, two monophasic and one recurrent neuromyelitis optica, several years after the diagnosis of MG, and discuss their clinical courses.

Miastenia gravis (MG) é doença autoimune caracterizada por episódios de fraqueza muscular alternados com melhora, causada por bloqueio da junção neuromuscular. Pacientes com MG podem apresentar outras doenças autoimunes, comumente hipo ou hipertiroidismo, e a associação de MG com doenças desmielinizantes é raramente descrita. Relatamos três pacientes brasileiros com MG que desenvolveram doenças desmielinizantes, dois monofásicos e um neuromielite óptica recorrente, vários anos após o diagnóstico de MG e discutimos seus cursos clínicos.

Avaliação clínica, endoscópica e manométrica da deglutição em pacientes com miastenia grave autoimune adquirida / Clinical, endoscopical and manometric evaluation of swallowing in patients with myasthenia gravis

Apresentamos 22 pacientes com diagnóstico de miastenia grave autoimune adquirida (MGAA), com queixa de disfagia orofaríngea, avaliados pela análise fonoaudiológica, nasofibrolaringoscópica e manométrica de esfíncter esofágico superior, com o objetivo de avaliar as fases do processo de deglutição e os aspectos anatômicos e funcionais do sistema estomatognático. A idade dos pacientes variou de 19 a 74 anos; 19 (86,4%) eram do sexo feminino e 3 (13,6%) do masculino. Os principais achados foram: (1) correlação entre avaliação fonoaudiológica e nasofibrolaringoscópica; (2) alterações do sistema estomatognático, em 100% dos casos; (3) desordens da mastigação / deglutição presentes em 100% dos pacientes, à avaliação fonoaudiológica e em 81,8%, à nasofibrolaringoscópica; (4) associação entre penetração/aspiração e episódios de pneumonia; (5) comprometimento muscular do sistema estomatognático, acúmulo de resíduos, escape precoce do bolo para a faringe e episódios de penetração/aspiração apresentaram correlação nítida com diminuição da contração faríngea. Estes achados justificam a necessidade de avaliar as fases do processo de deglutição em pacientes com MGAA, com o objetivo de propiciar uma orientação adequada e prevenir episódios de pneumonia aspirativa, desnutrição e desidratação, devido à administração inadequada de alimentação via oral

[Clinical, endoscopical and manometric evaluation of swallowing in patients with acquired autoimmune myasthenia gravis]. / Avaliação clínica, endoscópica e manométrica da deglutição em pacientes com miastenia grave autoimune adquirida.

Dysphagia, or difficulty swallowing, is a common problem following myasthenia gravis (MG) and may lead to aspiration of saliva, food or liquids. We herein present 22 MG patients, with complaint of dysphagia, evaluated by phonoaudiological evaluation, nasofibrolaryngoscopical analysis and manometry of upper esophageal sphincter. The main objective was to evaluate the phases of the swallowing process and anatomical and functional aspects of oropharyngeal musculature. The age of patients varied from 19 to 74 years; being 19 female and 3 male. The main data were: (1) statistically significant relation between the phonoaudiological clinical evaluation and nasofibrolaryngoscopical one; (2) stomatognatical system disorders present in 100%; (3) swallowing and chewing disorders present in 100%, when clinically evaluated, and in 81,8% when evaluated by the nasofibrolaryngoscopy; (4) statistically significant relation between penetration/aspiration and antecedents of pneumonia; (5) stomatognatical muscles very altered, difficulty controlling the bolus, collection of food in the pharynx or larynx and presence of penetration and/or aspiration showed significant correlation with weakness of a pharyngeal muscles. These findings justify the necessity to evaluate clinically the swallowing phases in MG patients, with the main purpose to prevent both aspiration and dehidration from inadequate oral intake.

Dosagens séricas repetidas de anticonvulsivantes em pacientes epiléticas / Repetitive serum dosages of anticonvulsants in epileptic patients

Para avaliaçäo da aderência ao tratamento em epilépticas crônicas estudamos 38 pacientes através de 144 dosagens séricas repetidas de anticonbulsivantes a intervalos semanais. Todas as pacientes apresentavam crises supostamente de dificil controle, isto é, tiveram crises no mês anterior à última consulta. O nível sérico da droga antiepiléptica estava abaixo da faixa terapêutica em 34// das amostras analisadas. Houve ainda variaçöes semanais importantes do nível terapêutico para subterapêutico das drogas e vice-versa. Baseados nestes achados sugere-se que a estratégia de dosagens séricas repetidas possa diferenciar as pacientes resistentes à droga daquelas que näo fazem uso regular do medicamento