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We report a child with biallelic COQ6 variants presenting with familial thrombotic microangiopathy (TMA). A Chinese boy presented with steroid-resistant nephrotic syndrome at 8 months old and went into kidney failure requiring peritoneal dialysis at 15 months old. He presented with hypertensive encephalopathy with the triad of microangiopathic haemolytic anaemia, thrombocytopenia, and acute on chronic kidney injury at 25 months old following a viral illness. Kidney biopsy showed features of chronic TMA. He was managed with supportive therapy and plasma exchanges and maintained on eculizumab. However, he had another TMA relapse despite complement inhibition a year later. Eculizumab was withdrawn, and supportive therapies, including ubiquinol (50 mg/kg/day) and vitamins, were optimized. He remained relapse-free since then for 4 years. Of note, his elder sister succumbed to multiple organ failure with histological evidence of chronic TMA at the age of 4. Retrospective genetic analysis revealed the same compound heterozygous variants in the COQ6 gene. [ABSTRACT FROM AUTHOR]

We present the case of a man in his 70s who developed acute confusion from hypertensive encephalopathy triggered by indomethacin. He was recently prescribed indomethacin, a non‐steroidal anti‐inflammatory drug (NSAID) for headaches. However, his headaches were in the context of worsening hypertension that was treated with trandolapril. The use of indomethacin consequently worsened his underlying condition. On presentation to the emergency department, his blood pressure was 190/110 mmHg. Bloodwork including electrolytes, glucose, metabolic studies, renal and liver function were within normal limits; infectious workup including blood and urine cultures subsequently returned negative; and brain computed tomography and magnetic resonance imaging revealed no acute process to explain his presentation. Indomethacin was discontinued and the patient's hypertension was treated with amlodipine. Both his confusion and underlying headaches resolved as his blood pressure normalized. The patient was diagnosed with hypertensive encephalopathy triggered by indomethacin. NSAID use can trigger blood pressure decompensation, especially in patients with underlying hypertension; this effect is particularly pronounced in patients treated with anti‐hypertensive medications that inhibit the renin‐angiotensin‐aldosterone (RAS) system. Symptomatic treatment with NSAIDs is not without potential harm; it is important to carefully consider a patient's underlying diagnosis, indication for therapy and risk for adverse effects. [ABSTRACT FROM AUTHOR]

Copyright of Neurologie Pro Praxi is the property of SOLEN sro and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

The most frequent cause of nephritic syndrome in the pediatric population is acute post-infectious glomerulonephritis (PIGN). A rare complication is posterior reversible encephalopathy syndrome (PRES), characterized by subcortical vasogenic cerebral edema associated with variable neurological symptoms. The development of autoimmune hemolytic anemia is an atypical clinical presentation. Objective: To report the coexistence of two unusual and serious extrarenal complications of PIGN and to discuss potential mechanisms involved in their development. Clinical Case: A 4-year-old male patient, with a 5-day history of hematuria and edema, headache, nausea, and vomiting. He was admitted in convulsive status and hypertensive crisis, laboratory showed C3 hypocomplementemia and high titers of Antistreptolysin O, which was interpreted as PIGN. Due to the presence of encephalopathy, PRES secondary to hypertensive emergency was suspected, which was confirmed by brain MRI. He also presented autoimmune hemolytic anemia, with hemoglobin up to 5 g/dL. The antihytreatment was based on antihypertensive therapy, neuroprotective measures, and steroid treatment. He was discharged 31 days after hospitalization and remained asymptomatic 6 months after discharge. Conclusions: There must be a high suspicion index of PRES before the appearance of nonspecific neurological symptoms during the evolution of a PIGN. The existing anemia on occasion can be autoimmune. [ABSTRACT FROM AUTHOR]

Objective: Challenging differential diagnosis Background: Pheochromocytoma, a rare catecholamine-secreting tumor, often presents with paroxysmal or sustained hypertension, tachycardia, headache, and diaphoresis. Timely diagnosis is essential to prevent adverse complications. Less common presentations include pheochromocytoma crisis, with severe neurological and cardiac complications. Case Report: We report a unique case of a 25-year-old woman who initially presented with pheochromocytoma-induced hypertensive encephalopathy and acute coronary syndrome. Echocardiography revealed takotsubo-like cardiomyopathy, and magnetic resonance imaging of the brain revealed posterior reversible encephalopathy syndrome. Initial treatment focused on controlling her blood pressure and supporting cardiac function. Due to her recovering from immediate crisis and absence of further symptoms, the patient refused further follow-up. However, she eventually experienced another episode of hypertensive crisis 2 years later. Subsequent investigations with 24-h urine tests revealed elevated vanillylmandelic acid levels (7.93 mg/24 h), normetanephrine (2638.72 µg/24 h), and nor-metanephrine to creatinine ratio (3546.67) and normal urine metanephrine levels (195.92 µg/24 h) and metanephrine to creatinine ratio (263.33). Contrast-enhanced computed tomography of the abdomen revealed a 4.3 x 3.1 x 4-cm mass in the right adrenal gland. A DOTATATE positron emission tomography scan revealed a 3.9 x 4.3 x 2.7-cm localized right adrenal pheochromocytoma. Biochemical testing and adrenal imaging revealed a previously undiagnosed pheochromocytoma. Following targeted medical therapy and right adrenalectomy, the patient achieved complete resolution of her hypertension and associated symptoms. Conclusions: Our case is a unique simultaneous presentation of posterior reversible encephalopathy syndrome and takotsubo- like cardiomyopathy, highlighting the importance to consider pheochromocytoma in acute neurological and cardiac presentations, even in the absence of typical symptoms. [ABSTRACT FROM AUTHOR]

Objective: Acute post-streptococcal glomerulonephritis (APSGN) is a common cause of acute glomerulonephritis in children. The condition may present as acute nephritic and/or nephrotic syndrome and rarely as rapidly progressive glomerulonephritis. ChatGPT (OpenAI, San Francisco, California, United States of America) has been developed as a chat robot supported by artificial intelligence (AI). In this study, we evaluated whether AI can be used in the follow-up of patients with APSGN. Methods: The clinical characteristics of patients with APSGN were noted from patient records. Twelve questions about APSGN were directed to ChatGPT 3.5. The accuracy of the answers was evaluated by the researchers. Then, the clinical features of the patients were transferred to ChatGPT 3.5 and the follow-up management of the patients was examined. Results: The study included 11 patients with an average age of 9.08±3.96 years. Eight (72.7%) patients had elevated creatinine and 10 (90.9%) had hematuria and/or proteinuria. Anti-streptolysin O was high in all patients (955±353 IU/mL) and C3 was low in 9 (81.8%) patients (0.56±0.34 g/L). Hypertensive encephalopathy, nephrotic syndrome, and rapidly progressive glomerulonephritis were observed in three patients. Normal creatinine levels were achieved in all patients. Questions assessing the definition, epidemiologic characteristics, pathophysiologic mechanisms, diagnosis, and treatment of APSGN were answered correctly by ChatGPT 3.5. All patients were diagnosed with APSGN, and the treatment steps applied by clinicians were similarly recommended by ChatGPT 3.5. Conclusions: The insights and recommendations offered by ChatGPT for patients with APSGN can be an asset in the care and management of patients. With AI applications, clinicians can review treatment decisions and create more effective treatment plans. [ABSTRACT FROM AUTHOR]

Hypertensive encephalopathy is a rare condition which is a clinically and radiologically diagnosed disorder. It usually presents in known hypertensive cases among young- and middle-aged adults. Although it presents with altered consciousness and other multisystem manifestations, symptoms of psychosis are rarely seen; management of the disorder is proper blood pressure control. Now, we present two cases of de novo hypertension and a case of chronic hypertension with no history of psychiatric illness and presented with symptoms of irritability, confusion, and hallucinations. To our knowledge, these are the few case reports showing psychosis in hypertensive patients presented with hypertensive encephalopathy. Categories: Internal Medicine, Psychiatry, Neurology

Introduction: Tacrolimus-associated neurotoxicity (TAN) manifests with wide clinical spectrum, ranging from mild tremors to severe encephalopathy. The isolated involvement of the brainstem is a rarely documented presentation of TAN, and its clinical and diagnostic characteristics are unclear. Methods: We report two cases of brainstem-isolated TAN (bi-TAN). Moreover, we performed a systematic review of the literature on bi-TAN and extracted data concerning demographics, clinical characteristics, radiological features, and management. The systematic literature search followed PRISMA guidelines and a pre-defined protocol. Results: Eleven patients, including our two, were identified (mean age: 41.3 years, ± 18.8; five males, 45%). Speech disturbance was the most common clinical presentation (45%). The mean latency from Tacrolimus initiation to bi-TAN onset was 26 days (± 30.8). Tacrolimus serum level tested above the reference range in three patients (mean: 26.83 ± 5.48). Brain MRI showed T2-FLAIR hyperintensities; three showed restricted diffusion on ADC maps. Neurological symptoms resolved completely in seven patients (63%) after Tacrolimus withdrawal or dose reduction. Conclusions: Our findings suggest that bi-TAN could represent a brainstem variant of posterior reversible encephalopathy syndrome. Recognition of bi-TAN as a potential cause of isolated brainstem lesions is crucial to disentangle the diagnostic work-up and ensure prompt withdrawal or reduction of the offending agent. [ABSTRACT FROM AUTHOR]

Background: Canine pheochromocytomas (PCCs) are rare tumors of the adrenal medulla. Clinical signs are often vague, resulting in intermittent catecholamine over secretion or neoplastic invasion of adjacent structures. Case Description: A 12-year-old Epagneul Breton dog with a 1-year history of chronic kidney disease, was examined for acute onset of severe neurological signs. Based on clinical and instrumental data, hypertensive encephalopathy was suspected. Cardiac and abdominal ultrasound were performed. Severe hypertensive cardiopathy and a right adrenal gland mass with invasion of the caudal vena cava were diagnosed. Computed tomography imaging confirmed the suspect of invasive malignant neoplasia. Emergency pharmacological therapy was started to reduce systemic pressure, improve clinical signs, and stabilize the dog in view of surgical resolution. After initial improvement, patient conditions abruptly worsened, and euthanasia was elected. Histology examination confirmed a right adrenal PCC, with caval invasion. Conclusion: To the authors' conclusions, acute hypertensive encephalopathy is a peculiar manifestation of PCCs. Ultrasound is a useful, and rapid test to suspect PCC as it can detect adrenal alterations, caval invasion, metastasis, and cardiac sequelae consistent with the condition. PCC can mimic multiple affections, and be misinterpreted, especially when a concurrent disease has already been diagnosed. Veterinarians need to be aware that comorbidities could mask clinical signs and delay diagnosis. Furthermore, this clinical case reminds us to include PCC also in the differential diagnosis of dogs with an acute onset of severe neurological signs. [ABSTRACT FROM AUTHOR]

BACKGROUND: This case report presents a history of familial hypomagnesemia with hypercalciuria and nephrocalcinosis (FHHNC). The patient was admitted to the hospital with hypertensive encephalopathy. FHHNC is a rare autosomal recessive disease caused by mutations in CLDN16 or CLDN19, resulting in insufficient magnesium and calcium kidney reabsorption. FHHNC manifestation starts in childhood, and over the years, its development leads to nephrocalcinosis and, consequently, chronic kidney disease (CKD), which is not slowed by routine administration of magnesium and thiazide diuretics. Ultimately, all FHHNC patients need kidney replacement therapy (KRT). CASE PRESENTATION: The patient was a 28-year-old male diagnosed with FHHNC and admitted to the emergency room due to hypertensive encephalopathy. The current situation was the patient's second hospitalization related to a hypertensive emergency caused by under-dialysis. Despite the signs of insufficient functioning of peritoneal dialysis (PD) (the primary chosen form of KRT), the patient refused the proposed conversion to hemodialysis (HD). Symptoms observed upon admission included disorientation, anxiety, and severe hypertension, reaching 213/123 mmHg. Due to his clinical condition, the patient was transferred to the intensive care unit (ICU), where the introduction of continuous veno-venous hemodiafiltration and hypotensive therapy stabilized blood pressure. Within the next few days, his state improved, followed by discharge from ICU. Eventually, the patient agreed to transition from PD to in-center HD. At the time, he was qualified for kidney transplantation, waiting for a compatible donation. CKD and dialysis are factors that significantly affect a patient's quality of life, especially in young patients with congenital diseases like FHHNC. CONCLUSIONS: For the aforementioned reasons, appropriate education and psychological support should be ensured to avoid the harmful effects of therapy non-compliance. [ABSTRACT FROM AUTHOR]

Background: Uraemia causes a generalised encephalopathy as its most common neurological complication. Isolated brainstem uraemic encephalopathy is rare. We report a case of fatigable ptosis and complex ophthalmoplegia in brainstem uraemic encephalopathy. Case presentation: A 22-year-old Sri Lankan man with end stage renal failure presented with acute onset diplopia and drooping of eyelids progressively worsening over one week. The patient had not complied with the prescribed renal replacement therapy which was planned to be initiated 5 months previously. On examination, his Glasgow coma scale score was 15/15, He had a fatigable asymmetrical bilateral ptosis. The ice-pack test was negative. There was a complex ophthalmoplegia with bilateral abduction failure and elevation failure of the right eye. The diplopia did not worsen with prolonged stare. The rest of the neurological examination was normal. Serum creatinine on admission was 21.81 mg/dl. The repetitive nerve stimulation did not show a decremental pattern. Magnetic resonance imaging (MRI) of the brain demonstrated diffuse midbrain and pontine oedema with T2 weighted/FLAIR hyperintensities. The patient was haemodialyzed on alternate days and his neurological deficits completely resolved by the end of the second week of dialysis. The follow up brain MRI done two weeks later demonstrated marked improvement of the brainstem oedema with residual T2 weighted/FLAIR hyperintensities in the midbrain. Conclusions: Uraemia may rarely cause an isolated brainstem encephalopathy mimicking ocular myasthenia, which resolves with correction of the uraemia. [ABSTRACT FROM AUTHOR]

Background: Generally infrequent, multiple sclerosis (MS) with late onset (LOMS) is characterized by an onset over the age of 50 and a mainly progressive course, while relapsing–remitting (RR) forms are less frequently observed and explored. This study aimed to characterize a large cohort of MS patients with RRMS at onset to assess the baseline factors related to the worst disability trajectories and explore the role of LOMS. Methods: The data were extracted from the Italian MS Register (IMSR). Disability trajectories, defined using at least two and up to twenty expanded disability status scale (EDSS) assessments annually performed, were implemented using group-based trajectory models (GBTMs) to identify different groups with the same trajectories over time. MS profiles were explored using multinomial logistic regression. Results: A total of 16,159 RR patients [1012 (6.26%) presented with LOMS] were analyzed. The GBTM identified four disability trajectories. The group with the most severe EDSS trend included 12.3% of the patients with a mean EDSS score > 4, which increased over time and exceeded 6 score. The group with medium severity EDSS trend comprised 21.9% of the patients and showed a change in EDSS > 3 scores over time. The largest group with 50.8% of patients reported a constant EDSS of 2 score. Finally, the benign group comprised 14.9% of the patients with a low and constant EDSS of 1 score over time. The probability of being in the worst groups increased if the patient was male; had LOMS or experienced brainstem, spinal, or supratentorial symptoms. Conclusions: Four MS severity profiles among RRMS patients in the IMSR have been reported, with LOMS being associated with a rapid worsening of EDSS scores. These findings have important implications for recognizing and managing how older age, aging, and age-related factors interact with MS and its evolution. [ABSTRACT FROM AUTHOR]

Objective: Rare disease Background: Acute necrotizing encephalopathy of childhood (ANEC) is a rare form of acute encephalopathy in children that is characterized by rapid and progressive deterioration in the patient's clinical condition. Our aim in reporting the case is to highlight the importance of early diagnoses and aggressive early management of ANEC to achieve a better outcome. We report the case of a 5-year-old girl who presented with acute progressive encephalopathy with bilateral symmetrical thalamic involvement and brainstem lesion proceeded by a flu-like illness. Case Report: We report a 5-year-old girl who was previously healthy. She presented with rapid progressive encephalopathy and status epilepticus following a flu-like illness. Her magnetic resonance imaging (MRI) showed significance symmetrical bilateral thalamic enhancement with brainstem lesions, which is consistent with the diagnosis of ANEC of childhood, and her initial electroencephalogram (EEG) showed severe encephalopathy. Despite extensive management of ANEC, the patient showed a poor prognosis. Conclusions: ANEC is a rare, specific, devastating condition in the pediatric population. Early diagnosis and aggressive management should be initiated immediately to avoid the high mortality associated with the condition. Furthermore, to highlight the importance of the clear history of the patient who presented with rapid and progressive deterioration in the level of consciousness and status epilepticus proceeded by viral illness, a rapid neuroradiological images such as brain MRI should be obtained to confirm the diagnosis of this rare condition. We also seek to increase awareness of this disorder among healthcare workers and general practitioners. [ABSTRACT FROM AUTHOR]

Introduction: Little is known about the longitudinal trajectory of brain growth in children with opsoclonus-myoclonus ataxia syndrome. We performed a longitudinal evaluation of brain volumes in pediatric opsoclonus-myoclonus ataxia syndrome patients compared with age- and sex-matched healthy children. Patients and Methods: This longitudinal case-control study included brain magnetic resonance imaging (MRI) scans from consecutive pediatric opsoclonus-myoclonus ataxia syndrome patients (2009-2020) and age- and sex-matched healthy control children. FreeSurfer analysis provided automatic volumetry of the brain. Paired t tests were performed on the curvature of growth trajectories, with Bonferroni correction. Results: A total of 14 opsoclonus-myoclonus ataxia syndrome patients (12 female) and 474 healthy control children (406 female) were included. Curvature of the growth trajectories of the cerebral white and gray matter, cerebellar white and gray matter, and brainstem differed significantly between opsoclonus-myoclonus ataxia syndrome patients and healthy control children (cerebral white matter, P =.01; cerebral gray matter, P =.01; cerebellar white matter, P <.001; cerebellar gray matter, P =.049; brainstem, P <.01). Discussion/Conclusion: We found abnormal brain maturation in the supratentorial brain, brainstem, and cerebellum in children with opsoclonus-myoclonus ataxia syndrome. [ABSTRACT FROM AUTHOR]

IgA vasculitis (IgAV), formerly known as Henoch–Scholein purpura, is a small vessel vasculitis, most commonly seen in pediatric patients, that can affect numerous internal organs including the kidneys, lungs, gastrointestinal tract, and the central nervous system (CNS). CNS manifestations of this condition include hypertensive encephalopathy, thrombosis, optic neuropathy, seizures, CNS vasculitis, and a more recently described phenomenon known as posterior reversible encephalopathy syndrome (PRES). Symptoms of PRES include hypertension, altered mental status, and seizures caused by vasogenic disruption of the blood–brain barrier, and the condition is diagnosed by characteristic edema‐related gray–white matter changes in the parieto‐occipital lobes on magnetic resonance imaging. Herein, we present a rare case of PRES as a presenting sign of IgAV to increase awareness about this unusual association. [ABSTRACT FROM AUTHOR]

Hypertensive encephalopathy is a rare condition which is a clinically and radiologically diagnosed disorder. It usually presents in known hypertensive cases among young- and middle-aged adults. Although it presents with altered consciousness and other multisystem manifestations, symptoms of psychosis are rarely seen; management of the disorder is proper blood pressure control. Now, we present two cases of de novo hypertension and a case of chronic hypertension with no history of psychiatric illness and presented with symptoms of irritability, confusion, and hallucinations. To our knowledge, these are the few case reports showing psychosis in hypertensive patients presented with hypertensive encephalopathy. Categories: Internal Medicine, Psychiatry, Neurology [ABSTRACT FROM AUTHOR]

Key Clinical Message: Posterior reversible encephalopathy syndrome may occur secondary to abrupt cessation of antihypertensive therapy. A gradual reduction in blood pressure and counseling regarding medication adherence are crucial to prevent adverse consequences. Posterior reversible encephalopathy syndrome (PRES) is a reversible clinical radiographic syndrome with headache, hypertensive encephalopathy, seizures, and visual disturbances as common modes of presentation. PRES can be attributed to several risk factors. We reported the case of a 66‐year‐old Asian female with PRES following nonadherence to antihypertensive treatment. Initially, her computed tomography scan of the head was normal. After 48 h, we again ordered a head CT scan, which showed lesions suggestive of hypertensive encephalopathy. We immediately reduced 20%–25% of mean arterial pressure, followed by a gradual blood pressure lowering to avoid adverse consequences. We did a follow‐up CT scan of the head at 2 weeks, showing the resolution of early lesions. Hence, we made a diagnosis of PRES. In these patients, it is crucial to ensure medication adherence to avoid complications. [ABSTRACT FROM AUTHOR]

Copyright of Revista Latinoamericana de Hipertension is the property of Revista Latinoamericana de Hipertension and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Background: The association of posterior reversible encephalopathy syndrome (PRES) and severe preeclampsia/eclampsia has been established but the frequency is uncertain. Objectives: To determine the frequency of PRES in severe preeclampsia or eclampsia. Methods: We searched published articles in PubMed, Cochrane library, Embase, and CINAHL from 1990 to 2020. We included articles that reported on six or more cases of PRES with eclampsia or severe preeclampsia who underwent neuroimaging during pregnancy or up to 6 weeks postpartum. Results: We identified 29 studies presenting data on 1519 women with eclampsia or severe preeclampsia. Among 342 women with eclampsia who had neuroimaging, 176 (51.4%) were diagnosed with PRES. Of 121 women with severe preeclampsia, 24 (19.8%) had PRES. The pooled maternal death rate was 5.3% (21/395). Conclusion: PRES is commonly reported on neuroimaging of women with eclampsia/ severe preeclampsia. The role of neuroimaging in eclampsia and especially in women with severe preeclampsia requires re-evaluation as further management is often dictated by this finding. [ABSTRACT FROM AUTHOR]

Background: Central variant of posterior reversible encephalopathy syndrome is clinico-radiological entity associated with hypertension, renal disease, cytotoxic immunosuppressive medications and autoimmune disease. We present a case of adult presented with uncontrolled hypertension and severe headache, unsteadiness of gait. Initial CT was done to rule of intracranial bleed showed hypodense area in pons. MRI finding showed vasogenic edema of brain stem leading to wide range of differential diagnosis. Follow up imaging within week shows marked resolution of MRI finding along with clinical improvement. This case represent importance of clinical and radiological corelation to diagnose central variant of posterior reversible encephalopathy syndrome, to avoid invasive investigation and treatment. It also emphasise the importance of awareness of this atypical central variant of PRES along with typical posterior cerebral variant. [ABSTRACT FROM AUTHOR]

Severe childhood hypertension is uncommon and frequently not recognised and is best defined as a systolic blood pressure (SBP) above the stage 2 threshold of the 95th centile + 12 mmHg. If no signs of end-organ damage are present, this is urgent hypertension which can be managed by the slow introduction of oral or sublingual medication, but if signs are present, the child has emergency hypertension (or hypertensive encephalopathy if they include irritability, visual impairment, fits, coma, or facial palsy), and treatment must be started promptly to prevent progression to permanent neurological damage or death. However, detailed evidence from case series shows that the SBP must be lowered in a controlled manner over about 2 days by infusing short-acting intravenous hypotensive agents, with saline boluses ready in case of overshoot, unless the child had documented normotension within the last day. This is because sustained hypertension may increase pressure thresholds of cerebrovascular autoregulation which take time to reverse. A recent PICU study that suggested otherwise was significantly flawed. The target is to reduce the admission SBP by its excess, to just above the 95th centile, in three equal steps lasting about ≥ 6 h, 12 h, and finally ≥ 24 h, before introducing oral therapy. Few of the current clinical guidelines are comprehensive, and some advise reducing the SBP by a fixed percentage, which may be dangerous and has no evidence base. This review suggests criteria for future guidelines and argues that these should be evaluated by establishing prospective national or international databases. [ABSTRACT FROM AUTHOR]

Introduction: Induced hypertension, administered peri-operatively during thoraco-abdominal aortic intervention, is one of the most effective methods to maintain spinal cord perfusion pressure. Posterior reversible encephalopathy syndrome or reversible cerebral vasoconstriction syndrome is a rare encephalopathy, possibly caused by excessive hypertension, usually encountered in the obstetric or cerebrovascular department. Report: A 61 year old man underwent open surgery for repair of an extent II dissecting thoraco-abdominal aneurysm. Several attempts at spinal drainage tube insertion one day prior to surgery failed. The Adamkiewicz artery was anastomosed by bypass, and transcranial motor evoked potentials were generally stable. Initially, no apparent neurological abnormality was observed after surgery; however, paraplegia occurred on post-operative day 1. The patient’s mean arterial pressure increased from > 85 mmHg to > 95 mmHg. His systolic blood pressure occasionally exceeded 170 mmHg. On post-operative day 3 he became blind. A serial imaging test revealed cerebral oedema of both posterior lobes and segmental constriction of the vertebral and basilar arteries. Posterior reversible encephalopathy syndrome with reversible cerebral vasoconstriction syndrome was diagnosed from the clinical context and imaging tests. Despite treatment with magnesium and calcium channel blockers, the patient’s visual acuity remained poor. Discussion: Excessive induced hypertension for spinal cord protection could rarely lead to cerebral vascular dysfunction, resulting in irreversible neurological damage. Awareness of this rare but devastating complication may help in early diagnosis, potentially mitigating permanent sequelae.

Objective: To assess the severity of CI in relation to the level of RDR to antihypertensive therapy in patients with primary arterial hypertension (AH) and the features of CI on the background of RAH. Methods: A complex of modern clinical, hemodynamic and neuropsychological methods was used to assess RDR to treatment and the severity of CI in groups of patients with high and very high RDR to treatment: the group I – with controlled arterial hypertension (CAH; n=40), the group II – with RAH (n=35). Results: When screening for CI on the Montreal Cognitive Assessment (MoCA) scale, a decrease in the average number of various domains of cognitive function in patients with CAH was found against the background of increased RDR to treatment compared with RAH. The results of the neurodynamic analysis of cognitive function in the Trail Making Test (TMT) indicate the relative sustain of selective attention and information processing speed. However, the established increase in the difference between the part B and part A performance in patients with AH demonstrated a decline in flexible thinking, cognitive control, and programming. The results of the study of regulatory functions showed that frontal dysfunction was observed more often in patients of group II, although the difference was statistically insignificant (p>0.05). Based on the results of speech studies, along with the results of memory impairment testing in the Clock Drawing (CDT) and Free and Cued Selective Reminding – Immediate Recall (FCSRT-IR) tests, a more pronounced decline of semantic memory was found in patients with RAH, indicating the possibility of memory impairment not only due to secondary neurodynamic changes but also due to the neurodegenerative processes. Conclusion: A significant decrease in semantic memory in patients with RAH suggests the possibility of the transformation of cerebral vascular lesions into neurodegenerative ones or their combination.

We report the pathological and molecular findings in an adult male Himalayan red panda (Ailurus fulgens fulgens) whose death was attributed to parenchymal brain haemorrhage (PBH) of the thalamus. Post-mortem examination revealed severe, acute PBH and intraventricular haemorrhage with major involvement of the thalamus, as well as scattered chronic microinfarctions. Vascular disease in the brain and other organs was suggestive of systemic hypertension. Histological lesions included arteriolar hyalinosis and varying degrees of arteriosclerosis, arterial tunica media hypertrophy and hyperplasia and infiltration of arterial walls by lipid-laden macrophages. Other relevant findings included marked myocardial fibrosis, lymphoplasmacytic tubulointerstitial nephritis, lymphoplasmacytic meningoencephalitis and chronic mitral valve degeneration. The changes in the cerebral vasculature were consistent with hypertensive encephalopathy and a cerebrovascular accident, specifically PBH, which has not been previously reported in this species. Additionally, polymerase chain reaction analysis for red panda amdoparvovirus (RPAV) was positive in the brain and kidneys. Preceded by hypertensive vascular changes and brain microinfarctions, sudden death in this animal likely resulted from fatal PBH with intraventricular haemorrhage. The clinicopathological role of RPAV infection is unknown in this case, although its contribution to the chronic renal disease is considered possible in the context of our current understanding of RPAV-associated pathology. [ABSTRACT FROM AUTHOR]

Background: Though epileptic seizures are common in posterior reversible encephalopathy syndrome (PRES), status epilepticus (SE) as the presenting feature is rare. Objective: To study the clinical spectrum and outcome of patients with SE as presenting feature of PRES. Methods: This is a retrospective study. PRES was diagnosed based on the clinical features and imaging findings on brain MRI (n = 40) which became normal after 6 months follow-up imaging. Patients with SE as the initial manifestation of PRES were identified. Baseline information regarding the clinical presentation, etiology, past history of illness, treatment history, imaging findings, EEG and long-term clinical outcome. Result: Seizure was the most common presentation seen in 31 patients (77.5%). The etiologies in PRES were preeclampsia, or eclampsia [n = 33 (82.5%)], hypertensive encephalopathy [n = 3 (7.5%)], systemic lupus erythematosus (SLE), AIP, and chronic renal failure (CRF) in one patient each [n = 01 (2.5%)]. Brain MRI showed the involvement of parieto-occipital lobes (n = 33 [82.5%]) mostly. Status epilepticus (generalized convulsive) was the presenting feature in eight cases (20%). Among them, five cases (0.5%) had a history of chronic epilepsy. In the remaining three patients, SLE and acute intermittent porphyria, CRF precipitated the SE. Conclusion: The study highlights the clinico-etiological spectrum of PRES and the identification of SE within its context leading to the early diagnosis and management if treated early. The role of antenatal care is important for the identification and treatment of etiologies, blood pressure, proper antiepileptic drug compliance and appropriate counseling. [ABSTRACT FROM AUTHOR]

Hypertensive crisis is rare in children. Among the rarest causes leading to this acute crisis, is bilateral adrenal tumour as a part of a syndrome, Von Hippel Lindau syndrome. The treatment is based on the excision of the adrenal tumour followed by long term surviellence. The authors present a case where conventional imaging with ultrasound and contrast enhanced CT scan demonstrated a right side adrenal tumour with raised catecholamines.The small left side tumour got missed on conventional imaging and got picked up on DOTA scan. The persistence of hypertension in post operative period can be related to such hidden functioning tumours. In view of the size of tumour & raised dopamine associated with high incidence of malignancy, robotic surgery was used for bilateral adrenal tumour excision which confirmed the diagnosis of Pheochromocytoma (PCC). [ABSTRACT FROM AUTHOR]

Cavernous malformation of brainstem is at increased risk of hemorrhage compared to supratentorial cavernomas. Increased morbidity associated with brainstem cavernoma(BCs) is defined by the inherent eloquence of the surrounding structures. At present watchful waiting, microsurgical resection and gamma knife surgery are the treatment modalities for symptomatic brainstem cavernoma. However, in the resource limited settings microsurgical excision by selecting an appropriate corridor can help in gross total removal in the meantime improving the neurological deficit and patient satisfaction. In this case series we have reviewed cavernomas of midbrain and pons, the technical difficulty with its management and its outcomes. [ABSTRACT FROM AUTHOR]

Central-variant posterior reversible encephalopathy syndrome is an atypical subtype of posterior reversible encephalopathy syndrome that occurs during rapid fluctuations in blood pressure, leading to cerebrovascular autoregulatory failure and endothelial dysfunction. Few reports have described posterior reversible encephalopathy syndrome in infants. A 4-month-old girl, who was diagnosed a month before with hypoxic ischemic encephalopathy due to sudden cardiac arrest, showed persistent renovascular hypertension with a systolic blood pressure of 200 mmHg. Computed tomography of the head revealed a new-onset low-attenuation area in the bilateral basal ganglia, and computed tomography of the trunk revealed severe long-segment narrowing of the abdominal aorta encompassing the bilateral renal arteries. She was treated with antihypertensive drugs and peritoneal dialysis. Follow-up imaging after blood pressure stabilization showed resolution of the low-attenuation area in the bilateral basal ganglia. We diagnosed her basal ganglia lesions as central-variant posterior reversible encephalopathy syndrome. She suffered from neurological sequelae attributable to hypoxic ischemic encephalopathy but showed no evidence of basal ganglia dysfunction. Here, we report a case of infantile central-variant posterior reversible encephalopathy syndrome involving bilateral basal ganglia lesions with mid-aortic syndrome. The differential diagnosis of infantile symmetric bilateral basal ganglia lesions is broad and includes genetic, acquired metabolic or toxic, infectious, inflammatory, vascular, and neoplastic pathologies. Among them, central-variant posterior reversible encephalopathy syndrome is rare but important because neurological prognosis may be favorable, and specific treatment, such as administration of antihypertensive drugs or discontinuation of drugs that induce posterior reversible encephalopathy syndrome, is possible.

The article "Scleroderma Renal Crisis: Clues From the Physical Exam" published in the Journal of Rheumatology discusses a case of a 56-year-old female presenting with acute encephalopathy and high blood pressure, leading to a diagnosis of scleroderma renal crisis (SRC). The patient showed physical signs such as foreshortened fingers and salt-and-pepper skin changes, along with laboratory findings of renal failure and thrombocytopenia. Treatment with losartan improved her condition, and further evaluation revealed positive Scl-70 antibodies and nonspecific interstitial pneumonia. The article emphasizes the importance of recognizing unique physical exam findings to promptly diagnose and treat SRC in patients with systemic sclerosis. [Extracted from the article]

Aim. To study brain magnetic resonance imaging (MRI) changes in patients with resistant hypertension (HTN) after renal denervation (RD) and its cerebral protection effectiveness.Material and methods. The study included 111 patients, of whom 50 were men (45%) and 61 were women (55%), who underwent RD. The mean number of antihypertensive drugs taken regularly was 4,1±1,1. All patients underwent brain MRI and 24-hour ambulatory blood pressure monitoring before and after renal denervation. The data were statistically processed using Statistica 10,0 software.Results. After RD, a pronounced antihypertensive effect was observed with a significant reduction in systolic and diastolic blood pressure by 10,3/5,5 and 13,1/7,3 mm Hg at 6 and 12 months, respectively. Office blood pressure, daytime, and nighttime blood pressure levels also decreased (p0,05).Conclusion. RD contributes to a significant decrease in blood pressure levels and exerts a cerebral protective effect by reducing lateral brain ventricles, subarachnoid spaces, and absence of ischemia progression and intracranial hypertension after intervention.

Epileptic seizures are defined as the transient appearance of signs or symptoms due to excessive or synchronous neuronal activity in the cerebral cortex. Pheochromocytomas and paragangliomas (PPGL) are tumors of chromaffin cells that arise from the medulla of the adrenal gland in 80­85% of patients and from the extra ­adrenal sympathetic tissue of the abdomen, pelvis and chest in 10­20% of patients. The clinical picture of PPGL is variable and ranges from the absence of symptoms to severe clinical picture, depending on the biochemical profile. They are most often manifested by paroxysmal hypertension, followed by episodes of severe headache or diaphoresis, while epileptic attacks are rare. Neurological symptoms are present in many patients with PPGL. Also, paroxysmal neurological conditions such as vasodilating headache, intracranial tumors, diencephalic ­autonomic epilepsy, hypertensive encephalopathy, focal arterial disease of the brain and anxiety state have been described, which may have similar clinical manifestations with pheochromocytomas. We present a 44­year­old woman, who has been diagnosed with pheochromocytoma as possible etiological basis of epileptic seizures. Pheochromocytoma, with its low incidence and “chameleon” clinical spectrum, should be considered as a potential etiological factor of convulsions. [ABSTRACT FROM AUTHOR]

Pheochromocytomas and paragangliomas are rare tumors. A 10 year‐old girl was brought to the emergency room with complaints of sudden vomiting and convulsions, and was initially diagnosed with hypertensive encephalopathy. Magnetic resonance imaging and computed tomography scan showed a large mass (6 × 3 × 3 cm) on the dorsal side of the inferior vena cava, surrounded by the right diaphragmatic crus, and closely attached to the aorta. Blood noradrenaline, urinary normetanephrine, and noradrenaline levels were elevated. The final diagnosis was retroperitoneal paraganglioma, then, surgery was contemplated. The location of the liver and great vessels in front of the tumor made the commonly performed transabdominal approach complicated. Therefore, retroperitoneoscopic surgery was preferred for safer resection, with better visualization in a sufficient space with less risk of damaging the surrounding organs. The retroperitoneoscopic approach is a good indication for tumors located behind the great vessels. [ABSTRACT FROM AUTHOR]

Background: Posterior reversible encephalopathy syndrome (PRES) can occur due to the detrimental effect of malignant hypertension on cerebral autoregulation. Most reported cases describe involvement of the supratentorial areas. Involvement of the posterior fossa structures in conjunction with supratentorial involvement has also been reported; however, PRES affecting the infratentorial structures without supratentorial involvement is a rare phenomenon. Clinical manifestations can involve severe headache, seizures, and reduced consciousness with treatment focused primarily on blood pressure control. Case Description: We report a case of PRES with isolated involvement of the infratentorial structures leading to obstructive hydrocephalus. The patient was managed with aggressive control of blood pressure and avoided ventriculostomy or posterior fossa decompression with a good outcome. Conclusion: Medical management in the absence of neurological deficit can be associated with a good outcome. [ABSTRACT FROM AUTHOR]

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Introduction: Systemic lupus erythematosis (SLE) is a multisystem disorder. Although Anti-Nuclear Antibody (ANA) positivity is described as an essential criterion of diagnosis in the 2019 EULAR guideline, ANA negative lupus is recognized up to 2% of all SLE patients who otherwise fulfil clinical and immunological criteria. Case Presentation: We describe a 20-year-old female diagnosed with ANA negative SLE who presented with renal SLE relapse and adult-onset seizures with high blood pressures; MRI was more in favour of cerebral lupus than posterior reversible encephalopathy syndrome, facilities to detect CSF anti ribosomal P antibodies were not available. During hospital stay, she developed severe bleeding manifestations with normal prothrombin and activated partial thromboplastin time, prolonged thrombin time and fibrinogen clauss with low normal fibrinogen levels which partially corrected with cryoprecipitate. Clot solubility was not performed. A diagnosis of acquired hypodysfibrinogenemia with possible factor X111 deficiency was made. Discussion: ANA-negative SLE is a clinical rarity. Differentiation between cerebral lupus and pure hypertensive encephalopathy may be challenging. Acquired coagulation disorders, which may exhibit diverse clinical and biochemical presentations, are well recognized in SLE.

Background and objective: The correlation (Rs > 0.7) of neutrophils expressing the dual endothelin1/signal peptide receptor (DEspR+CD11b+/CD66b+) with severity of hypoxemia (SF-ratio) and multi-organ failure (SOFA-score) in patients with acute respiratory distress syndrome (ARDS) suggest the hypothesis that the DEspR+ neutrophil-subset is an actionable therapeutic target in ARDS. To test this hypothesis, we conducted in vivo studies to validate DEspR+ neutrophil-subset as therapeutic target and test efficacy of DEspRinhibition in acute neutrophilic hyperinflammation models. Methods: We performed tests in lipopolysaccharide (LPS)-induced acute neutrophilic inflammation in three species - human, rhesus macaque, rat - with increasing dose-dependent severity. We measured DEspR+CD66b+ neutrophils in bronchoalveolar lavage fluid (BALF) in healthy volunteers (HVs) 24-hours after segmental LPS-challenge by ChipCytometry, and DEspR+CD11b + neutrophils in whole blood and BALF in an LPS-induced transient acute lung injury (ALI) model in macaques. We determined anti-DEspR antibody efficacy in vivo in LPS-ALI macaque model and in high-mortality LPS-induced encephalopathy in hypertensive rats. Results: ChipCytometry detected increased BALF total neutrophil and DEspR+CD66b+ neutrophil counts after segmental LPS-challenge compared to baseline (P =0.034), as well as increased peripheral neutrophil counts and neutrophil-lymphocyte ratio (NLR) compared to pre-LPS level (P <0.05). In the LPS-ALI macaque model, flow cytometry detected increased DEspR+ and DEspR[-] neutrophils in BALF, which was associated with moderate-severe hypoxemia. After determining pharmacokinetics of single-dose anti-DEspR [hu6g8] antibody, one-time pre-LPS anti-DEspR treatment reduced hypoxemia (P =0.03) and neutrophil influx into BALF (P =0.0001) in LPS-ALI vs vehicle mock-treated LPS-ALI macaques. Ex vivo live cell imaging of macaque neutrophils detected greater "intrinsic adhesion to hard-surface" in DEspR+ vs DEspR[-] neutrophils (P <0.001). Anti-DEspR[hu6g8] antibody abrogated intrinsic high adhesion in DEspR+ neutrophils, but not in DEspR[-] neutrophils (P <0.001). In the LPS-encephalopathy rat model, anti-DEspR[10a3] antibody treatment increased median survival (P =0.0007) and exhibited brain target engagement and bioeffects. Conclusion: Detection of increased DEspR+ neutrophil-subset in human BALF after segmental LPS-challenge supports the correlation of circulating DEspR+ neutrophil counts with severity measure (SOFA-score) in ARDS. Efficacy and safety of targeted inhibition of DEspR+CD11b+ neutrophil-subset in LPSinduced transient-ALI and high-mortality encephalopathy models identify a potential therapeutic target for neutrophil-mediated secondary tissue injury. [ABSTRACT FROM AUTHOR]

Background and Aim: Acute post-infectious glomerulonephritis (PIGN) can occur due to various etiologies. Among these, post-streptococcal glomerulonephritis is the common cause. Though the burden has drastically decreased over the years in developed nations, it remains a reason for concern in developing countries. This study aimed to document the burden, clinical presentation, etiology, and outcome of PIGN referred to a tertiary care center in a developing country. Methods: This retrospective study was conducted in a tertiary care teaching hospital in northeast India. All cases diagnosed with acute PIGN were included in the study. Cases with an alternate diagnosis and cases with incomplete records were excluded from the study. Data on relevant clinical, demographic, and laboratory variables were extracted from the case records and discharge summary. Simple descriptive statistics, such as frequency and proportion were used. Results: A total of 202 cases of PIGN were included in the study. The Mean±SD annual admission rate was 22.4±6.1 per year. The Mean±SD age at presentation was 10.0±3.9 years and the male to female ratio was 1.2 to 1. The most common clinical features at the time of presentation were hypertension in 183 patients (90.59%), edema in 168 (83.16%), history of oliguria in 146(72.27%), and hematuria in 168 patients (83.2%). Proteinuria was present in 95 cases (47.03%). Either clinical or serological evidence of preceding streptococcal infection was observed in 160 children (83.2 %). Two cases had scrub typhus and one case had hepatitis B seropositivity. Hypertensive encephalopathy and left ventricular failure were observed in 20(9.90%) and 44 children (21.78%), respectively. Admission to the pediatric intensive care unit was required in 28.21%. No mortality was observed. Conclusion: PIGN constitutes a significant burden in this part of India. The incidence of complications was high but the outcome was good with adequate acute care. [ABSTRACT FROM AUTHOR]

Posterior reversible encephalopathy syndrome (PRES) is characterized by transient vasogenic edema predominantly in supratentorial areas within the posterior circulation regions. Although PRES with only brainstem involvement is quite rare, accurate diagnosis is important because prompt antihypertensive therapy contributes to a favorable outcome. Herein, we report a case with isolated brainstem PRES showing dramatical improvement in an apparent diffusion coefficient (ADC) value of the lesion in magnetic resonance imaging (MRI) after clinical remission. The present case suggests the association between favorable clinical course and complete amelioration on MRI. [ABSTRACT FROM AUTHOR]

Although altered consciousness and other neurologic manifestations are frequently seen in hypertensive encephalopathy, behavioral and psychotic symptoms are rarely seen. We describe a patient with no previous psychiatric history who was admitted for hypertensive crisis. A few days after admission, his blood pressure remained uncontrolled and he started to exhibit episodes of confusion, agitation, and psychosis. During one particular episode, he overcame multiple staff members and physical restraints to bite off two of his fingers without any signs of pain. Brain computed tomography (CT) was notable for possible posterior cerebral and cerebellar edema. His confusion and agitation gradually resolved with successful blood pressure management. This is the first reported case of extreme, agitated behaviors and auditory hallucinations in a patient with hypertensive crisis.

Background and Objectives: Altered mental status (AMS) is a challenging diagnosis in elderly patients and has a wide range of etiologies. The aim of this study was to know the clinical and etiological profile in the elderly, so that physicians will be better aware of AMS backgrounds and reducing morbidity and mortality rates among the patients. Materials and Methods: This was a prospective observational study conducted at the emergency department (ED) and medical intensive care unit (MICU) of a tertiary care hospital in South India from November 1, 2019, to October 31, 2020. Patients aged 65 years and older who presented to the ED and MICU with AMS were included in this study. They were evaluated clinically and necessary investigations were done to know the etiologies. Results: Among 100 elderly patients, 64% belonged to young old (age group of 65–74 years), 30% belonged to old (age group of 75–84 years), and 6% belonged to oldest old (>85 years). Sixty‑one percent were male and 39% were female. The most common cause for AMS in elderly was primary neurological cause (40%, cerebrovascular accident was most common cause). The other causes were metabolic encephalopathy (29%, hyponatremia being most common) followed by multifactorial causes (24%). Infections and hypertensive encephalopathy were noted in 6% and 1% respectively. The common comorbidities found were hypertension (71%) and type 2 diabetes mellitus (50%), and 28% had diabetes mellitus and hypertension, whereas 25% had multiple comorbidities. Conclusion: The elderly patients who present with AMS must be screened for the above etiologies. This study helps the physicians to have an idea regarding common causes of AMS in elderly patients at presentation, hence appropriate management approach thus helps in reducing morbidity and mortality. [ABSTRACT FROM AUTHOR]

Pregnant women developing generalised tonic–clonic seizures in the absence of a prior neurological disorder are often diagnosed as eclamptic. Posterior reversible encephalopathy syndrome (PRES) is a distinct neuroimaging condition associated with long-term sequelae, which may occur in pregnancy. Some reports suggested PRES to be the pathophysiological process leading to eclampsia, whereas others observed PRES and eclampsia to have varying clinical severity and risk factors. In this case–control study, risk factors associated with PRES were compared to those for eclampsia in women with hypertension presenting with seizures who had undergone neuroimaging. PRES was noted to occur in 22.5% (51/227) hypertensive pregnant women presenting with seizures that otherwise would have been classified as eclampsia. An additional 51 women with eclampsia underwent neuroimaging. Women who had PRES had higher systolic (155.3 vs 144.5, p = 0.04), diastolic (99.2 vs 93.4, p = 0.006) and mean (117.9 vs 110.4, p = 0.001) blood pressure at admission compared to those with eclampsia. Eclampsia and PRES may occur through a similar pathophysiological mechanism, resulting in the same spectrum of neurological complications of preeclampsia, with PRES being the severest form of the disease process. PRES is difficult to differentiate from eclampsia based on clinical and laboratory investigation, except for high blood pressures, without adjunctive MRI/CT neuroimaging. Future studies should assess the role of biomarkers as well as long-term neurological sequelae in pregnant women with a diagnosis of PRES. [ABSTRACT FROM AUTHOR]