BackgroundPOEMS syndrome is a rare multi-systemic disease characterized by polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes. Arterial or venous thrombosis is a less-common complication of POEMS syndrome. Ischemic stroke has also been reported sporadically. However, the association between POEMS syndrome and ischemic stroke has not been entirely understood.MethodsA case of ischemic stroke caused by cerebral vasculitis in a patient with POEMS syndrome was presented. Then a comprehensive review and analysis of the literature were performed.ResultsA total of 28 patients were identified. The common clinical manifestations of POEMS syndrome were rather non-specific in patients with ischemic stroke compared with those of patients without ischemic stroke. Twenty patients were found with multiple ischemic lesions (71.5%). In the 25 patients who had undergone the evaluation of cerebral arteries, nineteen patients (76.0%) were found with cerebral vasculopathy. Twelve patients (48.0%) had more than one cerebral artery involved. Ischemic events were documented in 8 patients even when they were undergoing all the therapy for ischemic stroke. Ten (55.6%) of the 18 patients who had survival data died within two years after stroke events.ConclusionComprehensive analysis of literature revealed several trends in patients with ischemic stroke and POEMS syndrome including a low survival rate and a preponderance of cerebral vasculopathy and multiple cerebral arteries affected. Ischemic stroke may be a poor outcome predictor in patients with POEMS syndrome. Further researches focusing on a larger cohort may help in better characterizing and treating this rare complication of POEMS syndrome.
Rationale:Perimesencephalic nonaneurysmal subarachnoid hemorrhage (PNSAH) is characterized by a pattern of extravasated blood restricted to the perimesencephalic cisterns, normal angiographic findings, and an excellent prognosis with an uneventful course and low risks of complication. The precise etiology of bleeding in patients with PNSAH has not yet been established. The most common hypothesis is that PNSAH is venous in origin. Intracranial venous hypertension has been considered as the pivotal factor in the pathogenesis of PNSAH. The underlying venous pathology such as straight sinus stenosis, jugular vein occlusion may contribute to PNSAH. We describe a patient in whom transverse sinus thrombosis preceded intracranial venous hypertension and PNSAH. These findings supported that the source of the subarachnoid hemorrhage is venous in origin.Patient concerns and diagnoses:A 45-year-old right-handed man was admitted to the hospital with a sudden onset of severe headache associated with nausea, vomiting, and mild photophobia for 6 hours. The patient was fully conscious and totally alert. An emergency brain computed tomography (CT) revealed an acute subarachnoid hemorrhage restricted to the perimesencephalic cisterns. CT angiography revealed no evidence of an intracranial aneurysm or underlying vascular malformation. Digital subtraction angiography of arterial and capillary phases confirmed the CT angiographic findings. Assessment of the venous phase demonstrated right transverse sinus thrombosis. Magnetic resonance imaging confirmed the diagnosis of cerebral venous sinus thrombosis (CVST). Lumbar puncture revealed an opening pressure of 360 mmH2O, suggestive of intracranial venous hypertension. Grave disease was diagnosed by endocrinological investigation.Interventions:Low-molecular-weight heparin, followed by oral warfarin, was initiated immediately as the treatment for cerebral venous sinus thrombosis and PNSAH.Outcomes:The patient discharged without any neurologic defect after 3 weeks of hospital stay. MR venography revealed recanalization of right transverse sinus at the 6-month follow-up. No clinical or neuroimaging evidence of relapse was detected at 12 months follow-up.Lessons:Hyperthyroidism may contribute to the development of CVST. The presence of acute transverse sinus thrombosis, as a cause of PNSAH, provides further support for the hypothesis that the source of PNSAH is venous in origin and intracranial venous hypertension plays a critical role in the pathogenesis of PNSAH.
The purpose of this study was to evaluate sympathetic skin response (SSR) and heart rate variability (HRV) in determining autonomic nervous system (ANS) involvement in patients with Parkinson disease (PD). Forty-eight idiopathic PD patients and 30 healthy controls participated in this study. SSR, HRV, Unified Parkinson's Disease Rating Scale (UPDRS) III, the Scales for outcomes in Parkinson's Disease-Autonomic (SCOPA-AUT), Hoehn and Yahr (H&Y) scale were evaluated. Absent lower limb SSR was determined unilaterally in 2, bilaterally in 1 of 3 advanced PD patients; there was significant difference between PD and control groups in terms of the SSR (P < 0.01), significant prolonged SSR latencies and decreased SSR amplitudes from bilateral hands and feet. Significant difference was noted in HRV between PD and control groups except for root mean square of successive differences (rMSSD) and high-frequency (HF) power (P < 0.05). There was a significant different correlation between the parameters of SSR and the SCOPA-AUT, and between the parameters (except HF power) of HRV and the SCOPA-AUT. Some parameters of SSR were relevantly associated with HRV. The right hand SSR amplitude correlated positively with the (SD) of all R-R interval, total spectral power, very low frequency. The right foot amplitude correlated positively with total spectral power. Both SSR and HRV parameters are sensitive in determining ANS dysfunction not only in late but also in the early stage of PD, which can be used for early detection of autonomic dysfunction in patients with PD and have the potential to serve as electrophysiological markers of dysautonomia of PD.
Background and purpose:The aim was to investigate the associations of haemorrhagic transformation (HT) and its clinical and radiological subtypes with functional outcome, mortality, early neurological deterioration (END) and neurological complications in patients with acute ischaemic stroke (AIS). Methods: A systematic review and meta-analysis of observational studies on the associations of overall HT, clinical HT subtypes (asymptomatic intracerebral haemorrhage [aICH] and symptomatic intracerebral haemorrhage [sICH]) or radiological HT subtypes (haemorrhagic infarction [HI-1 or HI-2] and parenchymal haemorrhage [PH-1 or PH-2]) with prognosis in patients with AIS was performed. PubMed, Web of Science and Embase were systematically searched. Random effects models were used to calculate pooled estimates.Results: Fifty-one studies with 100,510 patients were pooled in the meta-analysis.Overall HT was associated with worse functional outcome (odds ratio [OR] 2.12, 95% confidence interval [CI] 1.55-2.90), increased mortality (OR 1.87, 95% CI 1.52-2.30), END (OR 2.35, 95% CI 1.46-3.77), early-onset seizures (OR 2.58, 95% CI 1.63-4.10) and poststroke epilepsy (OR 2.23, 95% CI 1.11-4.49). For clinical subtypes, sICH remained significantly associated with the aforementioned poor prognoses except post-stroke epilepsy, and aICH was associated with worse functional outcome but was unrelated to mortality.For radiological subtypes, PH (especially PH-2) was strongly associated with poor prognosis. HI-2 was associated with worse functional outcome, and HI-1 was associated with a lower risk of mortality and END.Conclusions: Regardless of whether AIS patients undergo thrombolysis or thrombectomy, overall HT, sICH and PH (especially PH-2) are associated with a substantially increased risk of worse functional outcome, mortality, END or neurological complications. The presence of aICH is related to worse functional outcome but is independent of increased mortality. HI-2 impairs functional independence, and HI-1 does not cause neurological impairment.
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