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Neuroradiology Jul 2024Neurofibromatosis type 1 (NF1) is a multisystem neurocutaneous disorder. Scoliosis and dural ectasia are features of the associated mesodermal dysplasia. Lateral...
Neurofibromatosis type 1 (NF1) is a multisystem neurocutaneous disorder. Scoliosis and dural ectasia are features of the associated mesodermal dysplasia. Lateral thoracic meningoceles can develop in NF1 and progressively enlarge due to cerebrospinal fluid (CSF) pulsations. Large meningoceles can cause compressive symptoms in the thorax. We are reporting a case of a NF1 presenting with acute onset respiratory distress, who also had chronic orthostatic headaches. CT chest showed unruptured enlarging bilateral lateral thoracic meningoceles causing lung compression. MRI of the brain and spine showed features of CSF hypotension, explaining the headaches. CSF hypotension with unruptured meningoceles is extremely rare. Management of the condition is challenging since surgical removal is prone to complications due to underlying mesodermal abnormalities. Cystoperitoneal shunting to relieve lung compression may worsen CSF hypotension. A shunt with a programmable valve allowed controlled drainage and successfully relieved lung compression without worsening of orthostatic headaches in our case.
Topics: Humans; Neurofibromatosis 1; Meningocele; Magnetic Resonance Imaging; Tomography, X-Ray Computed; Male; Female; Intracranial Hypotension; Hypotension; Thoracic Vertebrae; Diagnosis, Differential; Rare Diseases
PubMed: 38755334
DOI: 10.1007/s00234-024-03381-4 -
Journal of Neurosciences in Rural... 2024Spontaneous intracranial hypotension (SIH) is a condition characterized by orthostatic headache associated with nausea, vomiting, tinnitus, vertigo, hypoacusis, neck...
Spontaneous intracranial hypotension (SIH) is a condition characterized by orthostatic headache associated with nausea, vomiting, tinnitus, vertigo, hypoacusis, neck pain/stiffness, and photophobia. Usual treatment includes bed rest, hydration, caffeine, analgesics, epidural blood patch, steroids, fibrin glue (N-butyl-cyanoacrylate), and surgical repair. In this series, we report two cases, who presented to us with features of SIH and were managed successfully with sphenopalatine ganglion block. This is a novel modality of management of SIH and has not been reported before.
PubMed: 38746504
DOI: 10.25259/JNRP_30_2024 -
Cureus Apr 2024Atrioventricular (AV) block is a common cardiac conduction disorder that is frequently encountered in clinical practice; however, the association with rare systemic...
Atrioventricular (AV) block is a common cardiac conduction disorder that is frequently encountered in clinical practice; however, the association with rare systemic conditions such as transthyretin amyloidosis (ATTR) is heavily underdiagnosed. ATTR amyloidosis is a systemic disorder characterized by the deposition of abnormal transthyretin protein fibrosis in various organs including the heart and vasculature, resulting in progressive organ dysfunction. We present a rare case of high-grade AV block unveiling ATTR cardiac amyloidosis with unusual hemodynamics, specifically severe supine hypertension with severe orthostatic hypotension. These findings posed a diagnostic challenge, underscoring the importance of a comprehensive diagnostic approach and meticulous review of medical history. Following pacemaker placement and the diagnosis of ATTR cardiac amyloidosis, our patient was started on a Tafamidis regimen.
PubMed: 38741795
DOI: 10.7759/cureus.58166 -
Journal of Rehabilitation and Assistive... 2024Early mobilisation is paramount in the rehabilitation of patients with acquired brain injuries. However, the effectiveness of mobilisation to standing combined with...
INTRODUCTION
Early mobilisation is paramount in the rehabilitation of patients with acquired brain injuries. However, the effectiveness of mobilisation to standing combined with passive leg movement in mitigating orthostatic intolerance remains uncertain. Hence, we investigated whether participants exhibited better tolerance standing in a motorized standing device with passive leg movements, Innowalk Pro, compared to a traditional standing frame.
METHODS
17 patients with acquired brain injury (<1 year post-injury) performed two sessions in each standing device on four separate days. Maximum standing time was 30 min, less when symptoms of syncope or volitional exhaustion occurred. Besides total standing time, electromyography of thigh muscles, and changes in mean arterial pressure and heart rate were monitored at rest and during standing.
RESULTS
No significant differences were found in standing time, changes in mean arterial pressure or heart rate between standing in Innowalk Pro and the standing frame. However, participants had significantly more thigh muscle activation ( = 0.006) when standing in Innowalk Pro.
CONCLUSIONS
Mobilising participants with a subacute acquired brain injury in a standing frame with motorised passive movements of the lower limbs did, despite higher thigh muscle activation, not lead to better orthostatic tolerance or prolonged standing time compared to a traditional standing frame.
PubMed: 38737723
DOI: 10.1177/20556683241240488 -
Parkinsonism & Related Disorders May 2024Therapeutic education programs are effective in several chronic conditions. However, evidence is lacking in multiple system atrophy (MSA). We aimed to assess efficacy...
BACKGROUND
Therapeutic education programs are effective in several chronic conditions. However, evidence is lacking in multiple system atrophy (MSA). We aimed to assess efficacy and safety of a comprehensive therapeutic education program in people with MSA (PwMSA) and their caregivers.
METHODS
In this prospective longitudinal study we included 16 PwMSA and their main caregivers in 4 groups of 4 dyads each. The program consisted of eight 60-min interdisciplinary sessions: introduction, orthostatic hypotension, speech therapy, gait and respiratory physiotherapy, psychological support, urinary dysfunction, occupational therapy/social work. UMSARS, NMSS, PDQ39, EQ5 and Zarit scales were administered at baseline and 6 months later. After each session participants filled-out a modified EduPark satisfaction questionnaire and a Likert scale. Educational material was generated for each session after suggestions by participants.
RESULTS
At baseline PwMSA and caregivers were comparable in age and sex, with significant correlation between UMSARS-IV (disability) and PDQ39 (quality of life). Adherence to sessions was of 94,92 %. Total modified EduPark scores and Likert scales did not differ in PwMSA vs. caregivers, mild-moderate vs. severe-advanced cases or between genders. The significant difference in satisfaction across sessions (p = 0.03) was driven by higher scores in speech, respiratory and occupational therapy sessions. Longitudinally there was no significant worsening in any scale, nor a significant increase post-vs. pre-program in the number of consultations.
CONCLUSIONS
The healthcare education program in MSA was feasible, satisfactory, and safe for patients and caregivers. The educational material of the program is being forwarded to incident MSA cases attending our clinic.
PubMed: 38735163
DOI: 10.1016/j.parkreldis.2024.106993 -
Medicine May 2024We investigated the correlation of orthostatic hypotension (OH) in Parkinson disease (PD) with the disease course and severity, and its possible impact on quality of... (Observational Study)
Observational Study
We investigated the correlation of orthostatic hypotension (OH) in Parkinson disease (PD) with the disease course and severity, and its possible impact on quality of life. 171 PD patients were recruited and divided into the PD-NOH (n = 91) and PD-OH groups (n = 80). Clinical data were collected. The severity and quality of life of PD patients were evaluated. The impact of disease severity was analyzed using logistic regression analysis. The ROC curve was plotted. There were significant differences (P < .05) between PD-NOH and PD-OH groups in terms of the disease course, non-motor symptoms (somnipathy), Hoehn&Yahr stage, LEDD score, RBDSQ score, PDQ-39 score, MMSE score, MoCA, MDS-UPDRS Part III scores during off- and on-periods, and NMSS score. Hoehn&Yahr stage (OR 4.950, 95% CI 1.516-16.157, P = .008) was closely associated with the risk of OH in PD. PDQ-39 score (OR 1.079, 95% CI 1.033-1.127, P = .001) in PD patients with OH further decreased. Patients with PD-OH experienced severe impairment in 4 dimensions of quality of life, including motor function, cognitive function, physical discomfort, and activities of daily living. Different clinical symptoms of PD-OH were positively correlated with PDQ39 subscales. The area under the ROC curve of the Hoehn&Yahr stage in predicting the occurrence of OH was 0.679 (95% CI 0.600-0.758), and that of the Hoehn&Yahr stage combined with levodopa equivalent dose, and MDS-UPDRS Part III score during off-period was 0.793 (95% CI 0.727-0.862). Higher Hoehn&Yahr stage is associated with increased risk of OH in PD patients, and deteriorated quality of life of PD patients. Patients with different OH symptoms are affected in different dimensions of their quality of life. The Hoehn & Yahr stage can independently predict the risk of OH in PD patients.
Topics: Humans; Parkinson Disease; Quality of Life; Hypotension, Orthostatic; Male; Female; Aged; Middle Aged; Severity of Illness Index; Disease Progression
PubMed: 38728450
DOI: 10.1097/MD.0000000000038169 -
Current Urology Reports Jul 2024Stress urinary incontinence (SUI) is a commonly observed condition in females, as well as in males who have undergone prostatectomy. Despite the significant progress... (Review)
Review
PURPOSE OF REVIEW
Stress urinary incontinence (SUI) is a commonly observed condition in females, as well as in males who have undergone prostatectomy. Despite the significant progress made in surgical techniques, pharmacotherapy has not yielded substantial outcomes within the clinical domain. This review aims to present a comprehensive overview of the existing pharmacotherapy options for stress urinary incontinence (SUI) and the emerging therapeutic targets in this field.
RECENT FINDINGS
One meta-analysis demonstrated that α-adrenergic medications are more efficacious in improving rather than curing SUI symptoms. One trial showed reduced pad weight gain with PSD-503, a locally administered α-adrenergic receptor agonist. New data show that duloxetine's risk outweighs its benefits. One small-scale trial was found to support the use of locally administered estriol in improving subjective outcomes. Emerging targets include serotonin 5HT agonists, selective inhibitors of norepinephrine uptake, and myostatin inhibitors. Only one of the evaluated drugs, duloxetine, has been approved by some countries. Currently, trials are evaluating novel targets. Systemic adverse effects such as gastrointestinal upset with duloxetine and orthostatic hypotension with α-adrenoceptor agonists have hampered the efficacy of drugs used to treat SUI in women and men.
Topics: Humans; Urinary Incontinence, Stress; Duloxetine Hydrochloride; Female; Male
PubMed: 38727982
DOI: 10.1007/s11934-024-01205-9 -
GeroScience May 2024This study aimed to examine the effects of intensive antihypertensive treatment (AHT), i.e., systolic blood pressure target ≤ 140 mmHg, on cerebral blood flow,...
This study aimed to examine the effects of intensive antihypertensive treatment (AHT), i.e., systolic blood pressure target ≤ 140 mmHg, on cerebral blood flow, cerebral autoregulation, and orthostatic hypotension, in a representative population of frail older adults. Fourteen frail hypertensive patients (six females; age 80.3 ± 5.2 years; Clinical Frailty Scale 4-7; unattended SBP ≥ 150 mmHg) underwent measurements before and after a median 7-week AHT targeting SBP ≤ 140 mmHg. Transcranial Doppler measurements of middle cerebral artery velocity (MCAv), reflecting changes in cerebral blood flow (CBF), were combined with finger plethysmography recordings of continuous BP. Transfer function analysis assessed cerebral autoregulation (CA). ANCOVA analysed AHT-induced changes in CBF and CA and evaluated non-inferiority of the relative change in CBF (margin: -10%; covariates: pre-AHT values and AHT-induced relative mean BP change). McNemar-tests analysed whether the prevalence of OH and initial OH, assessed by sit/supine-to-stand challenges, increased with AHT. Unattended mean arterial pressure decreased by 15 mmHg following AHT. Ten (71%) participants had good quality TCD assessments. Non-inferiority was confirmed for the relative change in MCAv (95%CI: -2.7, 30.4). CA remained normal following AHT (P > 0.05), and the prevalence of OH and initial OH did not increase (P ≥ 0.655). We found that AHT in frail, older patients does not reduce CBF, impair autoregulation, or increase (initial) OH prevalence. These observations may open doors for more intensive AHT targets upon individualized evaluation and monitoring of hypertensive frail patients.Clinical Trial Registration: This study is registered at ClinicalTrials.gov (NCT05529147; September 1, 2022) and EudraCT (2022-001283-10; June 28, 2022).
PubMed: 38724874
DOI: 10.1007/s11357-024-01174-4 -
Journal of the Neurological Sciences Jun 2024Orthostatic hypotension (OH) is associated with an increased risk of dementia, potentially attributable to cerebral hypoperfusion. We investigated which patterns and...
BACKGROUND
Orthostatic hypotension (OH) is associated with an increased risk of dementia, potentially attributable to cerebral hypoperfusion. We investigated which patterns and characteristics of OH are related to cognition or to potentially underlying structural brain injury in hemodynamically impaired patients and healthy reference participants.
METHODS
Participants with carotid occlusive disease or heart failure, and reference participants from the Heart-Brain Connection Study underwent OH measurements, neuropsychological assessment and brain MRI. We analyzed the association between OH, global cognitive functioning, white matter hyperintensity (WMH) volume and brain parenchymal fraction with linear regression. We stratified by participant group, severity and duration of OH, chronotropic incompetence and presence of orthostatic symptoms.
RESULTS
Of 337 participants (mean age 67.3 ± 8.8 years, 118 (35.0%) women), 113 (33.5%) had OH. Overall, presence of OH was not associated with cognitive functioning (β: -0.12 [-0.24-0.00]), but we did observe worse cognitive functioning in those with severe OH (≥ 30/15 mmHg; β: -0.18 [-0.34 to -0.02]) and clinically manifest OH (β: -0.30 [-0.52 to -0.08]). These associations did not differ significantly by OH duration or chronotropic incompetence, and were similar between patient groups and reference participants. Similarly, both severe OH and clinically manifest OH were associated with a lower brain parenchymal fraction, and severe OH also with a somewhat higher WMH volume.
CONCLUSIONS
Severe OH and clinically manifest OH are associated with worse cognitive functioning. This supports the notion that specific patterns and characteristics of OH determine its impact on brain health.
Topics: Humans; Female; Hypotension, Orthostatic; Male; Aged; Magnetic Resonance Imaging; Brain; Middle Aged; Neuropsychological Tests; Hemodynamics; Cognition; White Matter; Heart Failure
PubMed: 38723328
DOI: 10.1016/j.jns.2024.123026 -
International Journal of Obstetric... Apr 2024Intracranial hypotension can cause persistent and severe orthostatic headaches. It is often the result of cerebrospinal fluid leakage secondary to iatrogenic causes. It...
Intracranial hypotension can cause persistent and severe orthostatic headaches. It is often the result of cerebrospinal fluid leakage secondary to iatrogenic causes. It can also happen spontaneously. Diagnosing and managing severe orthostatic headaches associated with intracranial hypotension can be challenging, particularly during late pregnancy. This case describes a parturient at 30 weeks' gestation who presented with severe headaches due to intracranial hypotension caused by dural defects and cerebrospinal fluid venous fistula. She was managed with serial non-targeted epidural blood patching as a bridging remedy. This allowed further fetal maturity before delivery and definitive management of the dural leakage after delivery.
PubMed: 38719763
DOI: 10.1016/j.ijoa.2024.103996