Moyamoya disease outcome

Moyamoya disease outcome

The disorder can lead to negative mood and stress, which, left unresolved, may increase adverse health outcomes. yang et al. conducted a cross-sectional survey to examine the stress and mood of adults with moyamoya disease. Participants were recruited at a university hospital in SeoulKorea. Data were collected through questionnaires and review of participants’ electronic medical records. A total of 109 adult patients participated. Significant correlations were found between perceived stress, anxiety, and depression. Adults with moyamoya disease experience anxiety, depression, and stress-related to the risk of cerebral hemorrhage or ischemia, similar to patients with other cerebrovascular diseases. If uncontrolled, negative mood and stress can cause adverse health outcomes. Health professionals caring for patients with moyamoya disease should carefully observe patients’ stress and mood and develop interventions tailored to stages of the disease to help patients manage stress and mood. The study results provide baseline information for understanding the level of and the factors associated with stress and mood 1).


Pediatric Moyamoya disease patients have greater patency and a greater ability to establish good leptomeningeal collateral circulation (LMC) status than adult patients, and poor LMC status has a strong correlation with severe clinical symptoms and poor postoperative outcomes. LMC status may be an important factor in the differences in clinical characteristics and prognosis between pediatric and adult MMD patients 2).


Recurrent stroke after surgical revascularization is still a big issue for moyamoya disease (MMD).

Female, left-sided surgery and edematous lesion were independent risk factors for postoperative TNEs; the left-sided surgery and edematous lesion were also independently associated with the severity of TNE. Although patients with postoperative TNEs had worse neurological status during the perioperative period, postoperative TNEs had no associations with worse mRS score at the time of discharge 3).


The outcome following surgery is very difficult to judge, and there is no standardised measurement to assess it. It is therefore important to know which approach for such patient is adequate.

Comparing to patients with acute idiopathic primary intraventricular hemorrhage (PIVH), patients with acute MMD-related PIVH have younger age, lower blood pressure, and better renal function. Moreover, patients with acute MMD-related PIVH have lower short-term mortality 4).


Sundaram et al., compared the long-term outcome of moyamoya patients treated conservatively to those who underwent RS.

A study population included all patients with moyamoya disease/syndrome from 2002 to 2012. The demographic, clinical characteristic and imaging details were reviewed. The outcome was obtained prospectively.

Of the 36 patients, 26 (72.2%) had MMD and 10 (27.8%) had moyamoya syndrome. The median age at onset of symptoms was 17.5 years (range, 10 months-55 years). Fifteen patients belonged to pediatric group and 21 were adults. All the pediatric patients had ischemic events at onset and 10 (47.6%) of the adults presented with hemorrhage. Twenty (55.6%) patients received conservative treatment and 16 (44.4%) underwent revascularization procedures. The median duration of follow-up was 28 months (range, 3-90 months). Three (18%) of the surgically treated patients had recurrent ischemic events on follow-up, but none of the conservatively treated patients had events. An excellent outcome (Modified Rankin Scale of ≤2) was seen in 12 (75%) surgically treated and 16 (94%) conservatively treated patients (p=0.17).

Compared to East Asians, our patients had a lower stroke recurrence rate and good functional outcome even with conservative treatment. Future studies should focus on clinical and imaging predictors of progression to select moyamoya patients for RS 5).

References

1)

Yang YS, Ryu GW, Yeom I, Shim KW, Choi M. Stress and Mood of Adults with Moyamoya Disease: A Cross-Sectional Study. Nurs Health Sci. 2020 Apr 26. doi: 10.1111/nhs.12729. [Epub ahead of print] PubMed PMID: 32336006.
2)

Liu ZW, Han C, Wang H, Zhang Q, Li SJ, Bao XY, Zhang ZS, Duan L. Clinical characteristics and leptomeningeal collateral status in pediatric and adult patients with ischemic moyamoya disease. CNS Neurosci Ther. 2020 Jan;26(1):14-20. doi: 10.1111/cns.13130. Epub 2019 Apr 13. PubMed PMID: 31875482.
3)

Lu J, Zhao Y, Ma L, Chen Y, Li M, Chen X, Ye X, Wang R, Zhao Y. Predictors and clinical features of transient neurological events after combined bypass revascularization for moyamoya disease. Clin Neurol Neurosurg. 2019 Aug 29;186:105505. doi: 10.1016/j.clineuro.2019.105505. [Epub ahead of print] PubMed PMID: 31622898.
4)

Yu Z, Guo R, Zheng J, Li M, Wen D, Li H, You C, Ma L. Comparison of acute moyamoya disease-related and idiopathic primary intraventricular hemorrhage in adult patients. World Neurosurg. 2019 Jan 24. pii: S1878-8750(19)30167-6. doi: 10.1016/j.wneu.2019.01.070. [Epub ahead of print] PubMed PMID: 30685378.
5)

Sundaram S, Sylaja PN, Menon G, Sudhir J, Jayadevan ER, Sukumaran S, Sreedharan SE, Sarma S. Moyamoya disease: a comparison of long term outcome of conservative and surgical treatment in India. J Neurol Sci. 2014 Jan 15;336(1-2):99-102. doi: 10.1016/j.jns.2013.10.014. Epub 2013 Oct 16. PubMed PMID: 24183032.

Aneurysmal Subarachnoid Hemorrhage Outcome in Elderly Patients

Aneurysmal Subarachnoid Hemorrhage Outcome in Elderly Patients

Aneurysmal subarachnoid hemorrhage outcome in elderly patients are at high risk for poor functional outcomes. However, among those presenting with good Hunt and Hess Stroke Scale scores, younger-elderly patients (ages 60-65 years) tend to fare better than older-elderly patients (ages >65 years). Elderly patients presenting with high-grade aSAH fare poorly regardless of age, which can inform clinical decision-making and prognostication 1).


The purpose of a study of Goldberg et al., from Inselspital, was to provide survival and outcome data to support clinicians making decisions on treatment for this subgroup of patients.

They performed a retrospective analysis of the Bernese SAH database for poor-grade (World Federation of Neurosurgical Societies grade IV and V) elderly patients (age ≥60 years) suffering from aSAH admitted to the institution from 2005 to 2017. Patients were divided into 3 age groups (60-69, 70-79, and 80-90 years).

Survival analysis was performed to estimate mean survival and hazard ratios for death. Binary logarithmic regression was used to estimate the odds ratio for favorable (modified Rankin Scale score of 0-3) and unfavorable (modified Rankin Scale score of 4-6) outcome. Results- Increasing age was associated with an increased risk of death after aSAH. The hazard ratio increased by 6% per year of age ( P<0.001; hazard ratio, 1.06; 95% CI, 1.03-1.09) and 76% per decade ( P<0.001; hazard ratio, 1.76; 95% CI, 1.35-2.29). Mean survival was 56.3±8 months (patients aged 60-69 years), 31.6±7.6 months (70-79 years), and 7.6±5.8 months (80-90 years). Unfavorable outcomes 6 to 12 months after aSAH were strongly related to an older age. The odds ratio increased by 11% per year of age ( P<0.001; odds ratio, 1.11; 95% CI, 1.05-1.18) and 192% per decade ( P<0.001; odds ratio, 2.92; 95% CI, 1.63-5.26). Conclusions- Risk for death and unfavorable outcome increases markedly with older age in elderly patients with poor-grade aSAH. Despite high initial mortality, treatment resulted in a reasonable proportion of favorable outcomes up to 79 years of age and only a small number of patients who were moderately or severely disabled 6 to 12 months after aSAH. Mean survival and proportion of favorable outcomes decreased markedly in patients older than 80 years 2).


It is also important to investigate the critical age for defining a higher risk population among elderly patients and the clinical grade at admission in order to provide a prognostic description and help guide the management of patients aged ≥ 70 years.

A retrospective study included 165 patients aged 70-90 years who underwent surgical or endovascular treatment for a ruptured aneurysm. In addition to medical and radiological data, telephone interviews were used to obtain the 1-year functional outcome.

Multivariate analysis revealed age (p = 0.001) and the World Federation of Neurological Surgeons (WFNS) grade (p = 0.001), regardless of the treatment modalities (surgical versus endovascular), as significant risk factors for a poor outcome, while a receiver operating characteristic analysis revealed 75 years as an appropriate cutoff value for the patient age to predict a poor 1-year functional outcome (area under the curve: 0.683). For the patients aged 70-75 years with good (1-3) and poor (4-5) WFNS grades, 81.9 % and 42.9 % achieved a favorable outcome (modified Rankin Scale 0-3), respectively, whereas for the patients over the critical age (> 75 years) with good and poor WFNS grades, 54.8 % and 5.9 % achieved a favorable outcome, respectively.

The long-term outcome for elderly patients with an aneurysmal SAH is affected primarily by the clinical condition at admission and the patient’s age in relation to the critical age (> 75 years), regardless of the treatment modalities, including surgical clipping and endovascular coiling 3).

References

1) 

Catapano JS, Louie CE, Lang MJ, DiDomenico JD, Whiting AC, Labib MA, Cole TS, Fredrickson VL, Cavalcanti DD, Lawton MT. Outcomes in a case series of elderly patients with aneurysmal subarachnoid hemorrhages in the Barrow Ruptured Aneurysm Trial (BRAT). World Neurosurg. 2020 Apr 15. pii: S1878-8750(20)30704-X. doi: 10.1016/j.wneu.2020.04.007. [Epub ahead of print] PubMed PMID: 32304888.
2) 

Goldberg J, Schoeni D, Mordasini P, Z’Graggen W, Gralla J, Raabe A, Beck J, Fung C. Survival and Outcome After Poor-Grade Aneurysmal Subarachnoid Hemorrhage in Elderly Patients. Stroke. 2018 Dec;49(12):2883-2889. doi: 10.1161/STROKEAHA.118.022869. PubMed PMID: 30571422.
3) 

Park J, Woo H, Kang DH, Kim Y. Critical age affecting 1-year functional outcome in elderly patients aged ≥ 70 years with aneurysmal subarachnoid hemorrhage. Acta Neurochir (Wien). 2014 Jun 21. [Epub ahead of print] PubMed PMID: 24950994.

Degenerative cervical myelopathy

Degenerative cervical myelopathy

The assessment, diagnosis, operative and nonoperative management of degenerative cervical myelopathy (DCM) have evolved rapidly over the last 20 years. A clearer understanding of the pathobiology of DCM has led to attempts to develop objective measurements of the severity of myelopathy, including technology such as multiparametric magnetic resonance imaging, biomarkers, and ancillary clinical testing. New pharmacological treatments have the potential to alter the course of surgical outcomes, and greater innovation in surgical techniques have made surgery safer, more effective and less invasive. Future developments for the treatment of DCM will seek to improve the diagnostic accuracy of imaging, improve the objectivity of clinical assessment, and increase the use of surgical techniques to ensure the best outcome is achieved for each individual patient 1).

Goel was troubled by the fact that his several PubMed and MEDLINE indexed articles on the subject published in leading journals dedicated to the study of the spine have not found any place in the huge reference list of 137 articles 2)

Definition

Epidemiology

Etiology

Pathophysiology

A review of Tetreault et al. summarizes current knowledge of the pathophysiology of DCM and describes the cascade of events that occur after compression of the spinal cord, including ischemia, destruction of the blood-spinal cord barrier, demyelination, and neuronal apoptosis. Important features of the diagnosis of DCM are discussed in detail, and relevant clinical and imaging findings are highlighted. Furthermore, this review outlines valuable assessment tools for evaluating functional status and quality of life in these patients and summarizes the advantages and disadvantages of each. Other topics of this review include epidemiology, the prevalence of degenerative changes in the asymptomatic population, the natural history and rates of progression, risk factors of diagnosis (clinical, imaging and genetic), and management strategies 3).

Clinical features

Patients may initially experience minimal symptoms 4) 5) but subsequently often develop pain, sensory deficits especially affecting their hands and feet, spasticity, imbalance, bladder symptoms, and experience frequent falls 6).

Diagnosing DCSM has traditionally relied on presence of clinical symptoms, including clumsy hands, paralysis of the lower extremities, gait disturbances, urinary/bowel incontinence and severe neurological dysfunction disturbances, urinary/bowel incontinence, and severe neurological dysfunction 7) 8).

Many people with cervical spondylosis or CSM are asymptomatic. However, patients with CSM are at higher risk of spinal cord injury (SCI) following minor injury.

Only a small percentage of people with spondylosis go on to develop symptoms consistent with cervical spondylotic myelopathy (CSM), which can cause significant and disabling neurological deficits, leading to loss of function, morbidity, and mortality.

In addition, diabetes mellitus (DM) is a frequent comorbidity for people of this age and may impact the severity of CCM.

Scales

European myelopathy score.

As a widespread used scale, the Modified Japanese Orthopaedic Association scale (mJOA) should be translated and culturally adapted 9).

see Cervical spine stenosis scales

Diagnosis

Treatment

Outcome

Randomized, controlled trials

A National Institutes of Health-funded (1R13AR065834-01) investigator meeting was held before the initiation of the trial to bring multiple stakeholders together to finalize the study protocol. Study investigators, coordinators, and major stakeholders were able to attend and discuss strengths of, limitations of, and concerns about the study. The final protocol was approved for funding by the Patient-Centered Outcomes Research Institute (CE-1304-6173). The trial began enrollment on April 1, 2014 10).

Case series

References

1)

Wilson JRF, Badhiwala JH, Moghaddamjou A, Martin AR, Fehlings MG. Degenerative Cervical Myelopathy; A Review of the Latest Advances and Future Directions in Management. Neurospine. 2019 Sep;16(3):494-505. doi: 10.14245/ns.1938314.157. Epub 2019 Aug 26. PubMed PMID: 31476852; PubMed Central PMCID: PMC6790745.
2)

Goel A. Degenerative Cervical Myelopathy. Neurospine. 2019 Dec;16(4):793-795. doi: 10.14245/ns.1938384.192. Epub 2019 Dec 31. PubMed PMID: 31905465.
3)

Tetreault L, Goldstein CL, Arnold P, Harrop J, Hilibrand A, Nouri A, Fehlings MG. Degenerative Cervical Myelopathy: A Spectrum of Related Disorders Affecting the Aging Spine. Neurosurgery. 2015 Oct;77 Suppl 4:S51-67. doi: 10.1227/NEU.0000000000000951. PubMed PMID: 26378358.
4)

Kovalova I, Kerkovsky M, Kadanka Z, Kadanka Z Jr, Nemec M, Jurova B, Dusek L, Jarkovsky J, Bednarik J. Prevalence and Imaging Characteristics of Nonmyelopathic and Myelopathic Spondylotic Cervical Cord Compression. Spine (Phila Pa 1976). 2016 Dec 15;41(24):1908-1916. PubMed PMID: 27509189.
5)

Martin AR, De Leener B, Cohen-Adad J, Cadotte DW, Nouri A, Wilson JR, Tetreault L, Crawley AP, Mikulis DJ, Ginsberg H, Fehlings MG. Can microstructural MRI detect subclinical tissue injury in subjects with asymptomatic cervical spinal cord compression? A prospective cohort study. BMJ Open. 2018 Apr 13;8(4):e019809. doi: 10.1136/bmjopen-2017-019809. PubMed PMID: 29654015; PubMed Central PMCID: PMC5905727.
6)

Davies BM, Mowforth OD, Smith EK, Kotter MR. Degenerative cervical myelopathy. BMJ. 2018 Feb 22;360:k186. doi: 10.1136/bmj.k186. Review. PubMed PMID: 29472200; PubMed Central PMCID: PMC6074604.
7)

Guan L, Chen X, Hai Y, et al. High-resolution diffusion tensor imaging in cervical spondylotic myelopathy: A preliminary follow-up study. NMR Biomed. 2017
8)

Sampath P, Bendebba M, Davis JD, et al. Outcome of patients treated for cervical myelopathy. A prospective, multicenter study with independent clinical review. Spine (Phila Pa 1976) 2000;25(6):670–76.
9)

Augusto MT, Diniz JM, Rolemberg Dantas FL, Fernandes de Oliveira M, Rotta JM, Botelho RV. Development of the Portuguese version of the modified Japanese Orthopaedic Association Score: cross-cultural adaptation, reliability, validity and responsiveness. World Neurosurg. 2018 Jun 1. pii: S1878-8750(18)31127-6. doi: 10.1016/j.wneu.2018.05.173. [Epub ahead of print] PubMed PMID: 29864576.
10)

Ghogawala Z, Benzel EC, Heary RF, Riew KD, Albert TJ, Butler WE, Barker FG 2nd, Heller JG, McCormick PC, Whitmore RG, Freund KM, Schwartz JS. Cervical Spondylotic Myelopathy Surgical Trial: Randomized, Controlled Trial Design and Rationale. Neurosurgery. 2014 Oct;75(4):334-346. PubMed PMID: 24991714.
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