Reversible Syndrome Research Articles

A case of primary hyperparathyroidism with a main manifestation of severe but reversible neurocognitive syndrome

A 65-year-old male was admitted to the emergency department with a suspected stroke, presenting with asymmetry of muscle strength and consciousness disorder. According to his family, the first signs had begun three months before hospitalization and had significantly worsened during the previous month. He had been fully independent and still working physically at the time of hospitalization. No head trauma, alcohol abuse, infections or past intoxications were reported. Single-phase computed tomography of the head revealed diffuse periventricular hypodensity and significant cortico-subcortical brain and cerebellum atrophy, with no signs of ischaemia or intracranial bleeding. Subsequently, the patient was admitted to the psychiatric ward, where behaviour disorganization, bradykinesia, massive memory disorders and somnolence were observed. During the first few days of hospitalization, the patient refused food intake did not comply with commands nor recognized his family members. However, no psychotic symptoms were observed. Due to tremors of the upper and lower limbs, increased muscle tension in the upper limbs and muscular weakness of the left arm, the patient was consulted neurologically and diagnosed with cerebellar syndrome of undetermined cause.

Impact of depressive symptoms and hepatic encephalopathy on health-related quality of life in cirrhotic hepatitis C patients.

Depression, common in chronic medical conditions, and hepatic encephalopathy (HE), a reversible neuropsychiatric syndrome due to liver dysfunction, are associated with impaired health-related quality of life (HRQOL) in cirrhosis and hepatitis C (HCV). This study investigated the impact of depression and HE on HRQOL in cirrhotic patients with HCV. A convenience sample of 43 ambulatory patients, with varying degrees of cirrhosis secondary to HCV, was prospectively enrolled in this study. Participants were assessed for any current depressive, fatigue, and daytime sleepiness symptoms and underwent a psychometric evaluation to determine the presence of HE symptoms. Participants reported current HRQOL on general health and liver disease-specific questionnaires. Diagnosis and current health status were confirmed via medical records. The associations between disease severity, depressive symptoms, HE, fatigue, and daytime sleepiness were measured. Predictors of HRQOL in this sample were determined. Depressive symptoms (70%) and HE (77%) were highly prevalent in this sample, with 58% actively experiencing both conditions at the time of study participation. A significant positive association was found between depressive symptoms and HE severity (P=.05). Depressive symptoms were significantly associated with fatigue (P<.001), daytime sleepiness (P<.001), general HRQOL (P<.001), and disease-specific HRQOL (P<.001). HE was significantly associated with fatigue (P=.02), general HRQOL (P<.001), and disease-specific HRQOL (P<.001). Depressive symptoms and HE were significant predictors of reduced HRQOL (P<.001), with depressive symptoms alone accounting for 58.8% of the variance. Depressive symptoms and HE accounted for 68.0% of the variance. Findings suggest a possible pathophysiological link between depression and HE in cirrhosis, and potentially a wider-reaching benefit of treating minimal and overt HE than previously appreciated.

Posterior reversible leukoencephalopathy syndrome in 17 year old girl

Objective - To describe a rare condition in children called posterior reversible leukoencephalopathy syndrome, its causes, diagnostic approach and treatment. Case report - A 17 year old girl experienced generalized seizures and head trauma after collapsing in the toilet. It was the first seizure in her life. Five days earlier she had given birth. Two more seizures followed in the next few hours after the first. Blood pressure measured at the time of the seizures was normal, but high afterwards. At that point she was admitted to the intensive care unit. Initially, a computed tomography scan was preformed and it revealed a pathologic finding. A magnetic resonance imaging (MRI) scan was performed to confirm the suspected diagnosis of posterior reversible leukoencephalopathy syndrome (PRES). During her stay in hospital other conditions were excluded and hypertension was considered as the cause of PRES. ACE inhibitors corrected the blood pressure. 12 days later her blood pressure and neurological status normalized, as well as the control MRI finding. Conclusion - Early recognition and intensive care are essential for a completely reversible outcome in children with PRES. In patients with hypertension, cytotoxic agent treatment or corticosteroid treatment, in case of the first seizure in their life, PRES should be suspected. Atypical MRI features can be expected in children, because the anterior watershed region may be as vulnerable as the vertebro-basilar system. In cases of hypertension, the most important issue is hypertension correction; hypertension should therefore be considered and corrected in treating seizures complicated by PRES.

Neurological Disorders and Pregnancy

Neurological disorders caused by pregnancy and puerperium include the posterior reversible encephalopathy syndrome, the amniotic fluid embolism syndrome (AFES), the postpartum angiopathy due to reversible vasoconstriction syndrome, and the Sheehan syndrome. Hypertension and proteinuria are the hallmarks of preeclampsia, seizures define eclampsia. Hemolysis, elevated liver enzymes and low platelets constitute the HELLP syndrome. Vision disturbances including cortical blindness occur in the posterior reversible encephalopathy syndrome (PRES). The Sheehan syndrome presents with panhypopituitarism post partum due to apoplexia of the pituitary gland in severe peripartal blood loss leading to longstandinghypotension. Some neurological disorders occur during pregnancy and puerperium with an increased frequency. These include stroke, sinus thrombosis, the restless legs syndrome and peripheral nerve syndromes, especially the carpal tunnel syndrome. Chronic neurologic diseases need an interdisciplinary approach during pregnancy. Some anticonvulsants double the risk of birth defects. The highest risk exists for valproic acid, the lowest for lamotrigine and levetiracetam. For MS interval treatment, glatiramer acetate and interferones seem to be safe during pregnancy. All other drugs should be avoided.

Understanding stress cardiomyopathy.

A 76-year-old woman loses a close friend. Several hours later, she has chest pain, diaphoresis, and shortness of breath. She presents to the emergency department, where an ECG shows deep T wave inversion across the precordium. Cardiac troponin is elevated. This presentation is classic for myocardial ischemia and for stress cardiomyopathy. Recognition of stress cardiomyopathy, and its distinction from acute coronary syndrome (ACS), is crucial for the clinician, since their pathophysiology and treatment are quite different. Stress cardiomyopathy is an acute reversible syndrome with characteristic wall motion abnormalities, consisting of apical hypokinesis or dyskinesis with sparing of the basal ventricular segments. Although the final outcome is generally good, early in their clinical course, patientsmay have cardiogenic shock (4 %), malignant ventricular arrhythmias (1–2 %), and death (1–1.5 %) [1]. Stress cardiomyopathy can complicate other conditions in the ICU, notably including subarachnoid hemorrhage. Patients with these more complex presentations often present clinical challenges. This manuscript aims to increase understanding of the identification and pathophysiology of stress cardiomyopathy to help practitioners manage this interesting syndrome.

Aneurysmal Subarachnoid and Spinal Hemorrhage Associated With Systemic Lupus Erythematosus

A 42-year-old woman with systemic lupus erythematosis (SLE), positive lupus anticoagulant, elevated titers of IgG and IgM anticardiolipin antibody and β2 glycoprotein antibodies, idiopathic thrombocytopenia, and hemolytic anemia presented with menorrhagia and anemia. She received both platelet and red cell transfusions and had an unrevealing endometrial biopsy. She was treated with intravenous immunoglobulin therapy for autoimmune-mediated thrombocytopenia and was taking hydroxychloroquine 200 mg twice daily that was a home medication. She was started on prednisone 60 mg daily. During her hospitalization, she developed a sudden, severe, sharp, and pounding occipital and nuchal headache with sharp shooting pains radiating down the back of the neck. She was alert and oriented with normal cranial nerve function, motor strength, and sensation. Her headache was unsuccessfully treated with fioricet and acetaminophen. The day after headache onset, a noncontrast head computed tomography revealed a subarachnoid hemorrhage (SAH) extending from the interpeduncular fossa caudally to the cervicomedullary junction (Figure 1). Computed tomographic angiogram of the head and neck revealed a focal left vertebral artery stenosis with poststenotic aneurysmal dilation of the intradural (V4) segment. There were additional multifocal areas of beading and long tapered vessel narrowing involving the M2 division of the left middle cerebral artery and anterior cerebral arteries and M3 division of the right middle cerebral artery (Figure 1). Magnetic resonance brain demonstrated multifocal but posteriorly predominant T2 hyperintense lesions that resembled posterior reversible leukoencephalopathy syndrome (Figure 2). Conventional angiogram confirmed the global small and medium vessel vasculopathy with a left V4 vertebral artery fusiform aneurysm measuring 10.6 mm×5.5 mm distal to the origin of the left posterior inferior cerebellar artery (Figure 2). Figure 1. Lupus-associated central nervous system vasculitis and subarachnoid hemorrhage (SAH). A , Computed tomographic (CT) head demonstrating perimedullary SAH (black arrow). B , CT angiogram demonstrating focal stenosis of the intracranial …

A Typical Case of Posterior Reversible Encephalopathy Syndrome Associated with Postpartum Eclampsia-HELLP Syndrome

Background: Posterior reversible encephalopathy syndrome (PRES) is a reversible syndrome characterized by seizures, headache, altered mentation, and loss of vision associated with white matter changes on imaging. Case: A 37-year-old multigravida woman had a severe, immediate postpartum eclampsia-HELLP syndrome (hemolysis, elevated liver enzyme levels, low platelet count) with PRES characterized by generalized seizures and altered mental status. Magnetic resonance brain imaging showed high-intensity lesions in non-posterior portions including the frontal lobe and cingulated gyrus, which resolved completely after 2 weeks along with complete symptom regression. Conclusions: Cases of postpartum PRES without involvement of posterior brain regions after eclampsia-HELLP syndrome are very rare. Patients with PRES do not always show typical manifestations. The importance of a prompt diagnosis is emphasized, as is the crucial role of rapid blood pressure reduction.

Unilateral mid-dilated reactive pupil after same session bilateral toric Implantable Collamer Lens implantation

We present a case of the unilateral mid-dilated reactive pupil to both light and near reflexes after uneventful refractive surgery of a healthy 32-year-old female patient who was diagnosed as a case of nonprogressive kertoconus (KC) and planned for surgical correction of astigmatism with toric Implantable Collamer Lens (tICL). On 1 st postoperative day, we noticed that there was anisocoria with right pupil being mid-dilated and left pupil of normal diameter. Both pupils react to light with the same degree of anisocoria in photopic and mesopic conditions. Intraocular pressure (IOP) was 14 mmHg in both eyes, although slight elevation of IOP occurred 1 h after surgery that had been self-limited 3 h postoperatively, and the patient was discharged without prescribing IOP lowering agents. Slit lamp examination revealed formed an anterior chamber with normal depth and cells +3 in the right eye and +2 in the left eye, ICL was well-centered in both eyes with normal vault and no posterior segment abnormalities were found. To our knowledge, this is the first case to be reported with the same findings after same session bilateral tICL implantation as a refractive surgery in nonprogressive KC patient. The patient was diagnosed with reversible Urrets-Zavalia syndrome (UZS) pupil in the right eye.

Rare Case of Posterior Reversible Leukoencephalopathy Syndrome Secondary to Acute Chest Syndrome.

We present a case of 29/m with a history of sickle cell disease who presented to the emergency department with sudden onset of chest, trunk, extremity, and back pain, consistent in quality and severity with the patient's usual pain crises. Soon after admission to the medical unit for acute chest syndrome (ACS), the patient developed sudden onset of hypertension associated with left sided hemiplegia, lethargy, dysarthria, aphasia, and left sided facial droop. Neuroimaging revealed that on MRI Brain there was multifocal extensive signal abnormality and a small focal areas of hemorrhage compatible with posterior reversible leukoencephalopathy syndrome (PRES). Patient was treated with levetiracetam and phenytoin and improved soon afterwards, with resolution seen on follow-up MRI two months later.

High Response Rate of Moxetumomab Pasudotox in Relapsed/Refractory Hairy Cell Leukemia Includes Eradication of Minimal Residual Disease: Potential Importance for Outcome

Abstract Background: In patients with hairy cell leukemia (HCL), the clinical importance of minimal residual disease is unknown; particularly by bone marrow aspirate (BMA) multicolor flow cytometry. It is often assumed that agents failing to eradicate MRD may prove equally effective as those that do. Moxetumomab pasudotox is a recombinant immunotoxin containing truncated Pseudomonas exotoxin fused to an anti-CD22 Fv. After the dose-escalation phase of a phase I trial in 28 patients with relapsed/refractory HCL treated with 5-50 ug/Kg every other day for 3 doses (QOD x3), a complete remission (CR) rate of 46% and overall response rate (ORR) of 86% were reported. Two cases of reversible grade-2 hemolytic uremic syndrome (HUS) were also reported, with transient grade I platelet and creatinine changes. Methods: To determine response and toxicity at a fixed dose, Twenty-one additional patients were enrolled at the highest dose level, totaling 33 patients at 50 ug/Kg QOD x3 and 49 patients overall. Patients required at least 2 prior courses of purine analog, or 1 course each of purine analog and rituximab if response duration to the first purine analog was &lt;2 years. CR criteria included absence of HCL cells by morphologic stains of bone marrow and blood. In CRs, multicolor flow cytometry was used to detect MRD in the BMA as well as blood. Results: No dose limiting toxicity or additional HUS cases were observed in any of the 202 cycles administered to 49 patients. Of 33 patients receiving 143 cycles of 50 ug/Kg QOD x3, the overall response rate was 88% and 21 (64%) achieved CR. Of 20 CRs followed for response duration, the median duration of CR (disease-free survival, DFS) was 42 mo (range 5 - 72), with 12 (60%) of 20 remaining in CR at a median of 42 mo. In addition to the 21 CRs achieved at 50 ug/Kg, there were 2 CRs out of 4 patients treated at 40 ug/Kg, and 5 CRs out of 12 patients treated at lower dose levels. Of the 28 CRs, MRD by BMA flow cytometry was evaluated in all except in 1 patient each at 50 and 40 ug/Kg, and 2 patients at lower doses. By BMA flow cytometry, MRD-negative CR was achieved in 11 (34%) of 32 at 50 ug/Kg, 1 of 3 at 40 ug/Kg, and 0 of 10 at lower doses (Fisher's Exact comparison above vs below 40 ug/Kg, p=0.041). MRD+ CRs included 9 at 50 ug/Kg, 0 at 40 ug/Kg, and 3 at lower doses. For the 12 MRD-negative CRs at 40-50 ug/Kg, DFS has not been reached, compared to a DFS of 17 mo in the 9 remaining MRD+ CRs at 40-50 ug/Kg (p&lt;0.0001). Of the 12 MRD-negative CRs, 11 (92%) remain in CR at 16-79 (median 42) mo of follow-up. Conclusion: Moxetumomab pasudotox can eliminate HCL MRD, and MRD eradication significantly correlated with CR durability. In HCL, particularly after multiple relapses, CRs should optimally be achieved without MRD. To our knowledge, moxetumomab pasudotox is the only known agent capable of eliminating MRD in HCL in a high percentage of patients without causing myelosuppressive toxicities common to chemotherapy. Its activity and safety profile support multicenter pivotal phase III testing, which is currently ongoing. This summary contains investigator reported data. This study was sponsored by MedImmune and supported by NCI's Intramural Research Program and the Hairy Cell Leukemia Research Foundation. Disclosures Lanasa: MedImmune: Employment. FitzGerald:NIH: Patents &amp; Royalties: Coinventor on NIH Patent. Pastan:NIH: Patents &amp; Royalties: Coinventor on NIH Patent.

Current state of knowledge on Takotsubo syndrome: a Position Statement from the Taskforce on Takotsubo Syndrome of the Heart Failure Association of the European Society of Cardiology.

Takotsubo syndrome is an acute reversible heart failure syndrome that is increasingly recognized in modern cardiology practice. This Position Statement from the European Society of Cardiology Heart Failure Association provides a comprehensive review of the various clinical and pathophysiological facets of Takotsubo syndrome, including nomenclature, definition, and diagnosis, primary and secondary clinical subtypes, anatomical variants, triggers, epidemiology, pathophysiology, clinical presentation, complications, prognosis, clinical investigations, and treatment approaches. Novel structured approaches to diagnosis, risk stratification, and management are presented, with new algorithms to aid decision-making by practising clinicians. These also cover more complex areas (e.g. uncertain diagnosis and delayed presentation) and the management of complex cases with ongoing symptoms after recovery, recurrent episodes, or spontaneous presentation. The unmet needs and future directions for research in this syndrome are also discussed.

Brimonidine Induced Anterior Uveitis

Purpose: To present the largest series reported so far of brimonidine induced granulomatous anterior uveitis.Methods: Retrospective chart review of patients with chronic glaucoma on treatment with brimonidine presenting with anterior uveitis.Results: 19 eyes of 12 patients with chronic glaucoma developed anterior uveitis with granulomatous keratic precipitates after being treated with brimonidine for periods of 7 days to 5 years (mean 19.7 months, +18.6 months). One patient had a gap of 1 year between onset in one eye and onset in the other. Ten eyes (52.6%) had concurrent granulomatous conjunctivitis; 16 eyes were pseudophakic. Stopping brimonidine led to complete, rapid resolution of uveitis in all patients, in most cases (11 eyes, 58%) without topical corticosteroids. No recurrences have occurred after withdrawal of brimonidine, over a follow-up period of 12–52 months (mean 27.9 months, +13.8 months).Conclusions: Ophthalmologists should be aware of this easily reversible uveitis syndrome that is induced by a common glaucoma medication. Familiarity with this entity is essential in making the correct diagnosis and avoiding unnecessary investigations and interventions.

Rotavirüs Gastroenterinde Posterior Reversible Lökoensefalopati Sendromu

Rotavirus is the primary cause of severe gastroenteritis in children. Generally, rotavirus infection is localized in the intestine, but there are some reports with extraintestinal involvement. It is occasionally associated with the central nervous manifestations. Herein, we report posterior reversible leukoencephalopathy syndrome in a 6-month-old boy with rotavirus gastroenteritis. (J Pediatr Inf 2015; 9: 126-9)

Difficulties encountered and solutions provided in the implementation of a multidisciplinary intervention for the prevention of dependency in the older population in rural areas: the MINDED study

Prevent increased number of dependent elderly has become a new priority for European public health. To achieve this, it is necessary to identify the frail older persons at risk of becoming dependent. Detecting frailty also allows to better understand an old patient's physiological reserves before making a therapeutic decision. To date, research tries to realize simple frailty screening tools allowing the clinician to better target - care provided to old adults during their first consultation. Furthermore, frailty is a reversible syndrome, so a global geriatric evaluation will allow the implementation of an individual program of early multi-domain care. The Multidomain intervention to prevent disability project in elders (MINDED), a randomized trial to prevent disability in elders, aims, in its second phase, to test the feasibility and to verify logistical, methodological and organizational aspects of the multi-domain intervention of the pilot study. In this paper, we shall describe the difficulties encountered and the solutions found during this feasibility phase.

Pseudotumor Cerebri in a Child With Familial Mediterranean Fever

Familial Mediterranean fever is an autosomal recessive polysystemic disease characterized by attacks of relapsing and self-limiting fever, peritonitis, pleuritis and arthritis. Neurologic involvement is rare but serious in Familial Mediterranean fever. Headache, seizures, demyelinating lesions, stroke, posterior reversible leukoencephalopathy syndrome, aseptic meningitis, convulsions, and cranial neuropathy have been reported in the literature. In this article, we present the first Familial Mediterranean fever and pseudotumor cerebri association in a child and discuss the pertinent literature.

Posterior reversible encephalopathy syndrome in children, epiphenomenon of a known or unknown disease

Posterior reversible encephalopathy syndrome, also known as reversible posterior leukoencephalopathy syndrome or reversible posterior cerebral edema syndrome, is a recently described disorder of the cerebrovascular autoregulation system with multiple etiologies, most of which cause acute hypertension. Its clinical symptoms include headache, decreased alertness, mental abnormalities such as confusion, diminished spontaneity of speech, and changed behavior, stupor, seizures, vomiting, and abnormalities of visual perception such as cortical blindness. Radiologic appearance consists in cortical/subcortical posterior cerebral artery territory lesions. We describe two cases of this disease in pediatric patients caused by different clinical conditions but supported by the same pathophysiology. Early recognition of posterior reversible encephalopathy syndrome as a complication during different diseases and therapies in childhood may facilitate precise diagnosis and appropriate treatment.

Posterior reversible encephalopathy syndrome in a child with Guillian-Barré syndrome

Posterior reversible encephalopathy syndrome (PRES) (synonym: reversible posterior leukoencephalopathy syndrome, reversible posterior cerebral edema syndrome) is a neurotoxic state associated with sudden elevation in blood pres- sure. It presents initially with lethargy and somnolence but can also manifest as headache, confusion, hemianopsia, nausea vomiting, seizures, vision disturbance, paresis, and cortical blindness. Very often these features are short lived and are resolved both clinically and radiologically but occasionally this disease can progress to coma and other devastating permanent complica- tions. Several etiological factors have been described in literature including hypertensive encephalopathy, preeclampsia, eclamp- sia, infection, immunosuppression, autoimmune diseases, cyclosporine toxicity, drug overdose, trauma with aortic dissection, pheochromocytoma and Guillain-Barre syndrome (GBS). Particularly, very few cases have been reported in literature indicating GBS as a precipitating factor for PRES. Therefore, we present here a brief review of literature describing association between GBS and PRES along with detailed diagnostic and therapeutic description. This review suggests necessity for adequate clinical suspicion for PRES in GBS afflicted patient followed by prompt diagnostic and therapeutic intervention. Failure to treat promptly and adequately might lead to the catastrophic consequence both in terms of morbidity and mortality.

POSTERIOR REVERSIBLE LEUKOENCEPHALOPATHY SYNDROME IN SYSTEMIC LUPUS ERYTHEMATOSUS PATIENTS

Posterior reversible leukoencephalopathy syndrome (PRES) is associated with severe neurological symptoms (headache, altered mental status, cortical blindness, focal neurological deficits, vomiting) and reversible changes on neuroimaging due to cerebral edema. Posterior reversible encephalopathy syndrome is mainly associated with hypertension, eclampsia, renal failure and/or use of immunosuppressive drugs. We present a literature review and a case report of PRES in a patients with systemic lupus erythematosus (SLE). The clinical symptoms and neuroimaging features were not specific. The peculiar role of SLE in the PRES development is not clear, since hypertension, renal diseases, immunosuppressive therapy and|or early management with high doses of intravenous steroids may facilitate PRES occurrence.

Fracaso renal agudo. Concepto, definición, epidemiologia, etiopatogenia y clasificación, manifestaciones clínicas, complicaciones y diagnóstico diferencial

Acute renal failure (ARF) is a reversible clinical syndrome caused by the sudden loss of renal function in early stages if the source of the damage is removed. It can be caused by pre-renal causes that compromise renal blood perfusion, which causes parenchymal or interstitial affect glomerular or tubular structures obstructive causes when the urinary excretion is affected. It assumes a common complication in critically ill patients and their conditions more severe forms high mortality, the need for alternative therapies and high health spending. Although the pathophysiology is variable, in case a large number of structural damage coexist level glomeruli and renal tubules. Clinical manifestations depend on the original etiology, including the elevation of nitrogenous products, and often impaired homeostasis, body composition, acid-base balance and catabolism.

Renal artery stenosis precipitates hyponatremic hypertensive syndrome and posterior reversible leukoencephalopathy.

Hyponatremic hypertensive syndrome (HHS) is an uncommon disorder usually encountered in the adult population with unilateral renal artery stenosis and is under-recognized in the pediatric population. A 19-month-old male presented with new-onset status epilepticus associated with neurological sequelae, and hypertension to a high of 248/150 mmHg. Lab work revealed significant hyponatremia, elevated peripheral renin activity, and increase in aldosterone and ADH levels. A diagnosis of HHS was made. Initial analysis revealed a high-grade proximal renal artery stenosis by magnetic resonance imaging (MRI) and angiogram. Electroencephalogram and an MRI of the brain demonstrated characteristic abnormalities of the left temporal-parietal regions consistent with posterior reversible leukoencephalopathy syndrome (PRES). The patient responded to right renal artery balloon dilation and stent placement. Since intervention and close blood pressure control with Amlodipine, the patient has been free of seizures and is neurologically intact. We report a case of malignant hypertension in a 19-month-old male secondary to renal artery stenosis with associated HHS and PRES. Prognosis of PRES in children with renal disease is excellent. Prompt intervention may offer near complete resolution of physiologic and symptomatic effects of HHS and PRES due to high-grade renal artery stenosis. This report was written with parental consent for de-identified case presentation and radiographs for the educational benefit of other medical professionals.