Central Venous Reflux, a Rare Cause of Neurological Manifestations in Hemodialysis Patients: A Case Report and Literature Review.

Central venous disease (CVD) is a serious complication in hemodialysis patients. Neurological manifestations are rare. We describe a female with end-stage renal disease with throbbing headache accompanied by paresthesia, weakness, and abnormal posture of her right hand during dialysis sessions. Motor symptoms completely resolved after each dialysis session, although the headaches persisted for several hours. No neurological deficit was evidenced on physical examination. Digital subtraction angiography identified an incomplete thrombosis of the left brachiocephalic vein with retrograde flow in the internal jugular vein, sigmoid sinus, and transverse sinus on the left side. This case illustrates that cerebral venous congestion due to CVD can produce neurological symptoms. Furthermore, we systematically review the literature to identify the characteristics of the cases described so far. This allows clinicians to know the entity and have a high index of suspicion in a hemodialysis patient who develops neurological symptoms.

Pseudotumoral Presentation of Cerebral Amyloid-Beta Angiopathy: Case Report and Review of Literature.

OBJECTIVE: Cerebral amyloid angiopathy-related inflammation (CAA-RI) is a rare and potentially treatable encephalopathy that usually affects people older than 50 years old and has an acute or subacute clinical presentation characterized by rapidly evolving cognitive decline, focal deficits and seizures. In a small subset of patients the disease can adopt a pseudotumoral form in the neuroimages that represents a very difficult diagnostic challenge. METHODS: Here in we report a patient with a tumour-like presentation of histopathologically confirmed CAA-RI. RESULTS: We also conducted a search and reviewed the clinical and radiological features of 41 cases of pseudotumoral CAA-RI previously reported in the literature in order to identify those characteristics that should raise diagnostic suspicions of the disease, there by avoiding unnecessary surgical treatments. CONCLUSION: The therapy of CAA-RI with steroids is usually effective and clinical and radiological remission can be achieved in the first month in approximately 70% of cases.

Convexity Subarachnoid Hemorrhage Secondary to Adalidumab in a Patient with Ulcerative Colitis.

The TNF-α antagonists are the drugs used for the treatment of ulcerative colitis (UC). Nontraumatic convexity subarachnoid hemorrhage is an infrequent nonaneurysmal subtype of subarachnoid bleeding caused mainly by reversible cerebral vasoconstriction syndrome (RCVS), cerebral amyloid angiopathy, and posterior reversible encephalopathy syndrome (PRES). We present a 26-year-old female patient with a diagnosis of UC taking Adalimumab. She received her last doses the same day she was admitted to our hospital for an acute severe UC exacerbation. Steroids were added to the treatment. Five days after admission she presented a thunderclap headache with photophobia, nausea, and vomiting. An MRI was performed showing left frontal convexity subarachnoid hemorrhage and hyperintense lesions on T2-weighted and FLAIR sequences located in both occipital lobes, left cerebellar hemisphere, and brainstem. Digital angiography was unremarkable. Adalimumab was discontinued but persisted on treatment with steroids. The patient evolved with complete resolution of her symptoms and was discharged with a normal neurological exam. Two months later, she was asymptomatic and her MRI revealed superficial siderosis secondary to cSAH with resolution of white matter hyperintensities. Convexity subarachnoid hemorrhage in our patient could be secondary to PRES or to RCVS. Analogous MRI findings can be observed in both syndromes, along with similar clinical and angiographic findings. This suggests that both conditions may reflect different manifestations of the same pathology, in which vascular tone and endothelial dysfunction play a major role. To our knowledge, this is the first report of a patient with severe UC and convexity subarachnoid hemorrhage associated with Adalimumab.

Prevalence of Fabry Disease in Young Patients with Stroke in Argentina.

BACKGROUND: Fabry disease (FD) is an underdiagnosed cause of stroke in young adults, but the frequency of this association is largely unknown. We estimated the prevalence of FD in a nationwide cohort of young adults who had stroke and transient ischemic attack (TIA) in Argentina. METHODS: This was a prospective, multicenter study of stroke and FD in young adults (18-55 years) conducted in Argentina between 2011 and 2015. Patients were enrolled if they had had a TIA or an ischemic or hemorrhagic stroke within the previous 180 days. FD was diagnosed by measuring α-galactosidase A activity (males) and through genetic studies (females). RESULTS: We enrolled 311 patients (54% men, mean age: 41 years). Ischemic events occurred in 89% of patients (80% infarcts, 9% TIA) and hemorrhagic strokes in 11%. One female (.3% of the total group, 1% of the cryptogenic ischemic strokes) had the pathogenic mutation c.888G>A/p.Met296Ile /Exon 6 on the GAL gene. Her only other manifestation of FD was angiokeratoma. Eighteen females had nonpathogenic intronic variations: c.-10C>T, c.-12G>A, or both. Two patients had the nonpathogenic mutation D313Y, while a third had the likely benign mutation S126G. CONCLUSIONS: FD was identified in 1 patient (.3%) in this first Latin American study. The patient presented with a late-onset oligo-symptomatic form of the disease. A large number of nonpathogenic mutations were present in our cohort, and it is essential that they not be mistaken for pathogenic mutations to avoid unnecessary enzyme replacement treatment.

Convexity subarachnoid hemorrhage: clinical features and etiology of an Argentinian cohort.

Nontraumatic convexity subarachnoid hemorrhage is an increasingly recognized subtype of subarachnoid bleeding. OBJECTIVE: Our aim was to describe the etiology and clinical features of a cohort of patients with convexity subarachnoid hemorrhage. METHODS: We retrospectively analyzed all cases of convexity subarachnoid hemorrhage admitted to our hospital between January 2012 and April 2017. Demographic features, clinical characteristics, complementary investigations, etiology and mortality were assessed. Twenty patients (65% females) were identified. Mean age: 53 years (range, 15-86 years). RESULTS: Symptoms on admission: headache (65%), sensory and/or motor symptoms (50%) and seizures (35%). Commonest causes: cerebral vein thrombosis (20%), reversible cerebral vasoconstriction syndrome (20%) and cerebral amyloid angiopathy (20%). Two patients died. CONCLUSION: Convexity subarachnoid hemorrhage may be related to a wide spectrum of etiologies. In our patients, an increased prevalence of cerebral vein thrombosis was observed. Mortality was low and not related to the bleeding itself.

Convexity subarachnoid hemorrhage: clinical features and etiology of an Argentinian cohort / Hemorragia subaracnóidea da convexidade: características clínicas e etiologia de uma coorte argentina

ABSTRACT Nontraumatic convexity subarachnoid hemorrhage is an increasingly recognized subtype of subarachnoid bleeding. Objective: Our aim was to describe the etiology and clinical features of a cohort of patients with convexity subarachnoid hemorrhage. Methods: We retrospectively analyzed all cases of convexity subarachnoid hemorrhage admitted to our hospital between January 2012 and April 2017. Demographic features, clinical characteristics, complementary investigations, etiology and mortality were assessed. Twenty patients (65% females) were identified. Mean age: 53 years (range, 15-86 years). Results: Symptoms on admission: headache (65%), sensory and/or motor symptoms (50%) and seizures (35%). Commonest causes: cerebral vein thrombosis (20%), reversible cerebral vasoconstriction syndrome (20%) and cerebral amyloid angiopathy (20%). Two patients died. Conclusion: Convexity subarachnoid hemorrhage may be related to a wide spectrum of etiologies. In our patients, an increased prevalence of cerebral vein thrombosis was observed. Mortality was low and not related to the bleeding itself.

RESUMO A hemorragia subaracnóidea não traumática da convexidade é um subtipo cada vez mais reconhecido de sangramento subaracnóideo. Objetivo: Nosso objetivo foi descrever a etiologia e as características clínicas de uma coorte de pacientes com hemorragia subaracnóidea da convexidade. Métodos: Foram analisados retrospectivamente todos os casos de hemorragia subaracnóidea da convexidade admitidos em nosso hospital entre janeiro de 2012 e abril de 2017. Foram avaliados os aspectos demográficos, características clínicas, investigações complementares, etiologia e mortalidade. Vinte pacientes (65% mulheres) foram identificados. Média de idade: 53 anos (intervalo, 15-86). Resultados: Sintomas na admissão: dor de cabeça (65%), sintomas sensitivos e/ou motores (50%) e convulsões (35%). Causas mais comuns: trombose venosa cerebral (20%), síndrome de vasoconstrição cerebral reversível (20%) e angiopatia amilóide cerebral (20%). Dois pacientes morreram. Conclusão: A hemorragia subaracnóidea da convexidade pode estar relacionada a um amplo espectro de etiologias. Em nossos pacientes, observou-se uma maior prevalência de trombose venosa cerebral. A mortalidade foi baixa e não relacionada à própria hemorragia.

Holmes tremor secondary to brainstem hemorrhage responsive to levodopa: report of 2 cases.

We report 2 patients who presented a brainstem hemorrhage and who, after 1 and 6 months, respectively, developed a 4-Hz postural and resting tremor consistent with Holmes tremor, which severely interfered with the activities of daily living. In both cases, levodopa dramatically improved the tremor. Pharmacological treatment of this condition is usually disappointing, and surgical procedures are commonly required for severe cases. Our patients, together with 13 others gleaned from the literature, suggest that in cases of Holmes tremor secondary to brainstem hemorrhage, levodopa can be a useful treatment, and it should be tested before considering invasive therapies.