Ware AL, Kulesz PA, Juranek J, Cirino PT, Fletcher JM. (2017) Cognitive control and associated neural correlates in adults with spina bifida myelomeningocele. Neuropsychology. 2017 May;31(4):411-423. doi: 10.1037/neu0000350. Epub 2017 Feb 16. PMID: 28206781

 

Abstract

OBJECTIVE:

Accelerated aging can occur in adult survivors of neurodevelopmental disorders, but has been narrowly studied in spina bifida myelomeningocele (SBM). Since discrete aspects of cognitive control and related neural network macrostructure deteriorate in normal aging, the specificity and trajectory of cognition and neuropathology incurred across adulthood in SBM were examined.

METHOD:

Adults (N = 120) with and without SBM completed working memory span and manipulation tasks, and an inhibitory control task. A subset (n = 53) underwent structural MRI. Effects of group, age, and their interaction on performance and select gray matter volumes were examined.

RESULTS:

Adults with SBM had significantly poorer working memory accuracy and overall inhibitory control performance than typical peers. Age negatively predicted inhibitory control. Group × Age significantly interacted on span accuracy; advanced age related to diminished performance in typical adults, but not in adults with SBM. SBM related to disproportionately enlarged cortical and putamen and reduced hippocampus volumes. Group × Age significantly interacted on cortical, but not subcortical gray matter volumes. Dorsolateral prefrontal, hippocampus, and putamen volumes negatively correlated with cognitive performance.

CONCLUSIONS:

Supporting previous literature, current findings elucidated a profile of executive impairment in SBM that was maintained in a parallel maturational trajectory to typical aging. Accelerated aging in cognitive control or subcortical gray matter was not supported in SBM. However, reductions in anterior and posterior cortical regions were exacerbated in older adults with SBM compared with typical peers. Overall results supported persistent anomalous neurodevelopmental maturation across the life span in SBM that related to diminished cognitive control. (PsycINFO Database Record

(c) 2017 APA, all rights reserved).

Perception of secondary conditions in adults with spina bifida and impact on daily life.

Wagner R, Linroth R, Gangl C, Mitchell N, Hall M, Cady R, Christenson M. (2015) Perception of secondary conditions in adults with spina bifida and impact on daily life.

Disabil Health J. 2015 Apr 4. pii: S1936-6574(15)00048-5. doi: 10.1016/j.dhjo.2015.03.012.

Abstract

BACKGROUND:

Spina bifida is a congenital defect of the neural tube resulting in motor and sensory disruption. Persons with spina bifida can also experience executive function impairments. Secondary conditions are physical, medical, cognitive, emotional, or psychosocial consequences to which persons with disabilities are more susceptible. Our experience suggested clinicians underappreciate the presence and impact of secondary conditions in adults with spina bifida because they do not specifically ask for this information.

OBJECTIVE:

Describe the presence and impact of secondary conditions on daily life, as perceived by adults with spina bifida.

METHODS:

A clinic-based sample was recruited from the active patient population of an adult specialty center for spina bifida-related care. All subjects were verbally administered a survey developed through literature review and clinical experience of the researchers. The survey measured the presence and perceived impact of secondary conditions. Recruitment and survey data collection occurred over a 6-month period to maximize age representation. Survey data were stratified by age, gender and lesion level for analysis.

RESULTS:

Seventy-two respondents completed the survey. Pain was commonly reported, along with pressure ulcers, bowel & bladder concerns, depression, sleep disturbance, and limited social and community participation. No significant relationships were found between the presence or perceived impact of secondary conditions and age, gender or level of lesion.

CONCLUSIONS:

Secondary conditions in spina bifida are present by early adulthood. Identifying these conditions during clinical encounters requires specific rather than general questions. Future study should evaluate earlier initiation of preventative measures by pediatric providers.
Copyright © 2015 Elsevier Inc. All rights reserved.

Health risk behaviors among young adults with spina bifida.

Soe MM, Swanson ME, Bolen JC, Thibadeau JK, Johnson N. (2012) Health risk behaviors among young adults with spina bifida. Developmental Medicine & Child Neurology. 2012 Sep 3.

Aim  Persons with spina bifida who adopt unhealthy lifestyles could be at increased risk of adverse health outcomes because the presence of spina bifida may magnify this risk. We estimated overall and age-specific prevalence of selected health risk behaviors (HRBs) in young people with spina bifida and examined the association between HRBs and depression. Method  We performed analyses on data obtained from individuals with spina bifida (n=130; mean age 23y SD 4y 5mo; 64 males, 66 females; 64% lumbosacral lesion; 77% with shunt) who participated in a population-based survey conducted by the Arkansas Spinal Cord Commission in 2005. Results  Compared with national estimates, young people with spina bifida tend to eat less healthy diets, do less exercise, and engage in more sedentary activities. Respondents were less likely to use substances (alcohol, tobacco, illegal drugs), which peaked among 25 to 31 year olds. About 90% saw a doctor in the previous year. Nearly one half reported mild or major depressive symptoms. In the logistic regression analysis after controlling for potential confounders (age, sex, ethnic group, education, employment, marital status, living arrangement, level of lesion, presence of shunt, mobility, self-rated health and healthcare utilization), major depressive symptoms were associated with current alcohol drinking (adjusted odds ratio: 4.74; 95% CI 1.18-19.04). Interpretation  Young adults with spina bifida exhibit unhealthy behaviors that continue into their late 20s. The findings highlight the need to increase awareness of their health risk profiles in the spina bifida community and show opportunities for mental health and health risk screening and counseling by healthcare providers.

 

PMID:22937873

Aggressive surgical management of patients with Chiari II malformation and brainstem dysfunction.

Rahman M, Perkins LA, Pincus DW. Aggressive surgical management of patients with Chiari II malformation and brainstem dysfunction. Pediatric Neurosurgery. 2009;45(5):337-44. 11.

BACKGROUND/AIMS:
Chiari II malformation represents a group of developmental abnormalities involving the caudal displacement of the cervicomedullary junction, pons, fourth ventricle, and medulla. This constellation of malformations is strongly associated with myelomeningocele (MM) and is a known cause of neurologic deterioration in older MM patients. We describe the evaluation and management of 4 adult MM patients who presented with brainstem compression and a retroflexed odontoid who were subject to aggressive surgical management including occipitocervical (OC) fusion with good clinical results.

METHODS:
Four MM patients who underwent OC fusion for new-onset brainstem dysfunction and myelopathy were identified in the practice of the principal investigator (D.W.P.) from 2003 to 2008.

RESULTS:
The initial evaluation of these patients included consideration of other diagnoses, such as hydrocephalus due to shunt malfunction and tethered cord. These patients were treated with aggressive surgical management. In some cases, multiple surgeries were performed. All 4 patients were treated with cervical decompression and OC fixation and initially had good outcomes. No patients required transoral decompression. Three of the 4 had stable improvement at their last follow-up appointment (mean follow-up of 9 months).

CONCLUSION:
Late deterioration in older MM patients may be secondary to brainstem or cervical spinal cord compression from ventral odontoid compression and Chiari II malformation, hydrocephalus, hydromyelia, or tethered cord. These patients may benefit from more than one surgery. Posterior decompression and OC fusion can avoid a morbid transoral odontoid resection, greatly improve patients' symptoms and prevent further neurologic decline.

PMID: 19907196

Pain, Especially neuropathic pain, in adults with spina bifida, and its relation to age, neurological level, completeness, gender and hydrocephalus

Werhagen L, Hultling C, Borg K. (2010)
Pain, Especially neuropathic pain, in adults with spina bifida, and its relation to age, neurological level, completeness, gender and hydrocephalus Journal of Rehabilitation Medicine. 2010 Apr;42(4):374-6.

Department of Clinical Sciences Karolinska Institutet, Division of Rehabilitation Medicine, Danderyd Hospital, Stockholm, Sweden. werhagen@hotmail.com
Abstract

STUDY DESIGN: Cohort study. OBJECTIVE: To investigate the prevalence of neuropathic pain in adults with spina bifida, to study the relationship between neuropathic pain, age at examination, gender, completeness of injury, neurological level and presence of hydrocephalus.

METHODS: A total of 110 patients with spina bifida who visited the spinal cord injury outpatient clinic Spinalis were included. At the yearly check-up they underwent examination by a physiotherapist and a neurologist and were interviewed about pain character, temporal profile and localization. The patients were divided into 2 groups; spina bifida with (n = 57) and without hydrocephalus (n = 53). Pain was classified as neuropathic when it was in an area of decreased sensibility and had no correlation to movement and/or inflammatory signs. Results were analysed by chi2 analysis and Fisher's exact test.

RESULTS: Twenty-two patients (20%) experienced nociceptive pain. Neuropathic pain was present in 11/110 (10%) patients, of these 62% experienced below level neuropathic pain. Neuropathic pain was present in 13% of male patients and 7% of female patients, 12% of patients with a lumbar level and 10% of patients with a thoracic level. Neuropathic pain was present in 9% of patients with a complete spinal cord injury, 14% of those with an incomplete spinal cord injury, 1,7% with hydrocephalus and 19% without hydrocephalus.

CONCLUSION: The results suggest that neuropathic pain is present in spina bifida. Careful analysis and classification of a patient's pain is clinically important. Neuropathic pain is more common in patients without hydrocephalus and in older patients. Presence of neuropathic pain was not related to gender, completeness of injury, or neurological level.


PMID: 20461341

Chronic headaches in adults with spina bifida and associated hydrocephalus

Edwards RJ, Witchell C, Pople IK. Chronic headaches in adults with spina bifida and associated hydrocephalus. European Journal of Pediatric Surgery. 2003 Dec;13 Suppl 1:S13-7.

INTRODUCTION: Adults with spina bifida and associated hydrocephalus are exposed to multiple risk factors for the development of chronic headache. The management of these patients can be complex and misdiagnosis can precipitate unnecessary shunt revision. This study aims to evaluate the usefulness of intracranial pressure (ICP) monitoring as a diagnostic tool in these cases and to look at the causes of chronic headaches and treatment outcomes for this patient population.

METHODS: All patients over the age of 18 years with a diagnosis of spina bifida and shunted hydrocephalus who had undergone inpatient or outpatient neurosurgical review within the last 10 years were identified in our hospital database. Case notes were then retrospectively reviewed to identify all patients who had undergone either inpatient or outpatient evaluation of chronic headaches (defined as a headache of at least one month's duration) occurring in the absence of any other symptoms or signs suggestive of raised intracranial pressure (ICP). The incidence, causes, management and outcome of chronic headache in these patients was determined.

RESULTS: 42 patients were identified, mean age 30 years (range 18 - 59). All had undergone lifelong follow-up. All had previously undergone shunt insertion for hydrocephalus. 16 had undergone endoscopic third ventriculostomy (ETV). 11 had undergone choroid plexus coagulation. 55 % (23/42) of patients underwent investigation for 1 or more episodes of chronic headache. Recurrent hydrocephalus due to shunt malfunction or ETV failure was excluded by ICP monitoring using either an intraparenchymal transducer or monitoring via a ventricular access device. All patients underwent repeat imaging, using CT and/or MR imaging. Identified causes of headache included: shunt blockage; shunt overdrainage; ETV failure and symptomatic Arnold-Chiari malformation. A history of choroid plexus coagulation (CPC) as an infant was associated with a decreased risk of chronic headache in later life (p = 0.02). In 8 patients no definite cause for headaches was identified, in 4 of these patients symptoms resolved spontaneously, the remainder required specialist pain management.

CONCLUSIONS: The aetiology of chronic headaches in this patient group is multifactorial. In the absence of other clinical symptoms or signs of raised ICP, ICP monitoring is an invaluable adjunct to management. 10 % of hydrocephalic adult spina bifida patients required specialist pain management for control of chronic idiopathic headache.

PMID: 14758561

Issues of medical management in adults with spina bifida

Related Articles, LinksMcDonnell GV, McCann JP. Issues of medical management in adults with spina bifida. Child's Nervous System. 2000 Apr;16(4):222-7.

The objective of this study was to establish the range of medical issues among those attending a clinic for adults with spina bifida (SB) and hydrocephalus (CASBAH). Owing to improvements in medical care in the past 3-4 decades, an increasing number of SB patients go on to prosper in adult life. Since 1990 there has been a CASBAH service in Belfast serving Northern Ireland on a regional basis. There are now 237 patients with SB remaining on the live register at CASBAH. All records were reviewed with regard to site of lesion, ambulatory ability, shunt placement and regularity of attendance. The records of 193 were also reviewed with reference to musculoskeletal problems, bladder function, bowel function, renal impairment and hypertension, skin breakdown, epilepsy and the incidence of clinically significant Chiari/hydrosyringomyelia. Of the patients on the register, 36% are wheelchair dependent, 8% have some ambulatory capacity but are largely wheelchair dependent, 22% are ambulatory with aid and 34% are independently ambulatory. Bladder function is normal in 8%, whilst 32% describe normal bowel function. Renal impairment is present in 48% of patients, and 15% are on anti-hypertensive therapy. Epilepsy is an active issue in 9%, and intraventricular shunts are in situ in 37% of patients. Scoliosis is present in 50% and 70% have joint deformities or contractures. Five patients have become symptomatic from the Chiari/hydrosyringomyelia complex. These data reflect the considerable range of disability in adult SB patients, the challenges presented in long-term management and the need for organised follow-up.

PMID: 10855520

Why do adults with spina bifida and hydrocephalus die?

McDonnell GV, McCann JP. Why do adults with spina bifida and hydrocephalus die? A clinic-based study. European Journal of Pediatric Surgery. 2000 Dec;10 Suppl 1:31-2.

AIM: To establish the death rate and factors contributing to death among attenders at a clinic for adults with spina bifida and hydrocephalus (CASBAH).

INTRODUCTION: Due to improvements in medical care in the past 40 years, increasing numbers of spina bifida patients go on to prosper in adult life. Despite this, levels of morbidity are high and services for such patients are uneven and unpredictable.

MATERIALS AND METHODS: Since 1990 there has been a CASBAH service in Belfast operating on a regional basis. Led by a consultant in rehabilitation medicine, it provides a multidisciplinary approach to patient care. Between 1990 and 1999 there have been 280 referrals to the clinic. Charts were reviewed regarding current health status and mode of death where relevant.

RESULTS: There have been 18 deaths during the term of the clinic (6.4%). There were four deaths due to renal failure and two due to cardiac failure. Two deaths occurred following surgery for a Chiari/hydrosyringomyelia complex and one patient died due to shunt malfunction. Three deaths were presumed related to infection. Two deaths were attributable to malignancy (carcinoma of a renal conduit and transitional cell carcinoma of the kidney) and other terminal events in one patient each were: basilar artery aneurysm rupture, post-partum pulmonary embolism, peptic ulcer disease, myocardial infarction and status epilepticus.

CONCLUSIONS: Although certain deaths were unpredictable during the period of review and not directly attributable to spina bifida, the majority reflect many of the related secondary health issues of the condition and indicate the need for organised, medically co-ordinated follow-up of adult patients.

PMID: 11214829

Dependence and perceived difficulty in activities of daily living in adults with cerebral palsy and spina bifida

Andren E, Grimby G. Dependence and perceived difficulty in activities of daily living in adults with cerebral palsy and spina bifida. Disability & Rehabilitation. 2000 May 10;22(7):299-307.

PURPOSE: The purpose of this study was to identify differences between two groups of subjects: one with cerebral palsy, the other with spina bifida in their dependence and their perceived difficulty in performing daily activities according to the Functional Independence Measure (FIM) and the Instrumental Activity Measure (IAM), and to compare these findings with the reported use of assistance.

METHOD: Community-living persons. 53 with cerebral palsy and 20 with spina bifida, aged 20 to 39 years, participated in semistructured interviews in their homes, where rating was performed using items from FIM and IAM.

RESULTS: Differences were found for the reported use of assistance and the dependence rated according to FIM and IAM. Significant differences for dependence were found between the CP and SB subjects concerning Eating, Bladder and Bowel items and for perceived difficulty concerning toileting, bladder and bowel. There was close overall agreement between dependence and perceived difficulty, except for the item walk/wheelchair.

CONCLUSIONS: Subjects in both groups needed help in basic and instrumental ADL. The ability of spina bifida subjects was more influenced in toileting, bladder, bowel than the cerebral palsy subjects and tended also to be so in mobility instrumental tasks. FIM and IAM do not cover all aspects of significance in community-living adults. Further items have to be developed, covering personal care and occupational as well as leisure domains.

PMID: 10877483

The transition from child to adult in neurosurgery

Vinchon M, Dhellemmes P. The transition from child to adult in neurosurgery.
Advances and Technical Standards in Neurosurgery. 2007;32:3-24.

The transition from child to adult is a growing concern in neurosurgery. Data documenting long-term follow-up are necessary to define this population's healthcare needs. In order to evaluate the problems posed by the child-to-adult transition in neurosurgery, we have studied the neurological, functional and social outcome of patients treated in our department for tumor of the central nervous system, hydrocephalus or myelomeningocele, and followed beyond the age of eighteen years. A large number of patients suffered from chronic ailments, either sequelae of their initial disease, or delayed complications of their initial treatment, with significant morbidity. The mortality during adulthood was 4.6% in the tumor group, 1.1% in the hydrocephalus group, and zero in the spina bifida group. The proportion of patients employed in normal jobs was 35.6, 18.7 and 11.5% for tumors, hydrocephalus and myelomeningocele respectively. IQ score and performance at school generally overestimated the capacity for social integration. Based on these data and on the available literature, we tried to identify the problems and devise solutions for the management of the transition from child-to-adulthood transition. Many problems present during childhood persist to adulthood, some of which are made more acute because of a more competitive environment, the lack of structures and inadequate medical follow-up. The transition from child to adult must be managed jointly by pediatric and adult neurosurgeons. More clinical research is required in order to precisely evaluate the problems posed by adult patients treated during childhood for the different neurosurgical diseases. Based on these data, a concerted trans-disciplinary approach is necessary, tailored to the specific needs of patients suffering from different diseases.

PMID: 17907472

What do we really know about the transition to adult-centered health care?

Binks JA, Barden WS, Burke TA, Young NL. What do we really know about the transition to adult-centered health care? A focus on cerebral palsy and spina bifida. Archives of Physical Medicine & Rehabilitation 2007 Aug;88(8):1064-73.

OBJECTIVES: To address the lack of synthesis regarding the factors, processes, and outcomes specific to the transition from child-centered to adult-centered health care for people with cerebral palsy (CP) and spina bifida (SB); more specifically, to identify barriers, to outline key elements, to review empirical studies, and to make clinical and research recommendations.

DATA SOURCES: We searched Medline and CINAHL databases from 1990 to 2006 using the key words: transition, health care transition, pediatric health care, adult health care, health care access, health care use, chronic illness, special health care needs, and physical disability. The resulting studies were reviewed with a specific focus on clinical transition for persons with CP and SB, and were supplemented with key information from other diagnostic groups.

STUDY SELECTION: All studies meeting the inclusion criteria were included.

DATA EXTRACTION: Each article classified according to 5 criteria: methodology, diagnostic group, country of study, age group, and sample size.

DATA SYNTHESIS: We identified 149 articles: 54 discussion, 21 case series, 28 database or register, 25 qualitative, and 34 survey articles (some included multiple methods). We identified 5 key elements that support a positive transition to adult-centered health care: preparation, flexible timing, care coordination, transition clinic visits, and interested adult-centered health care providers. There was, however, limited empirical evidence to support the impact of these elements.

CONCLUSIONS: This review summarizes key factors that must be considered to support this critical clinical transition and sets the foundation for future research. It is time to apply prospective study designs to evaluate transition interventions and determine long-term health outcomes.

PMID: 17678671

Adult tethered cord syndrome in patients with postrepair myelomeningocele: an evidence-based outcome study.

George TM, Fagan LH. Adult tethered cord syndrome in patients with postrepair myelomeningocele: an evidence-based outcome study. Journal of Neurosurgery. 2005 Mar;102(2 Suppl):150-6.

OBJECT: As patients with myelomeningocele age, tethered cord syndrome (TCS) in adults with postrepair myelomeningocele has become more common. The authors have structured an evidence-based review of the literature for the purpose of addressing the following questions. (1) How is the diagnosis made? (2) What is the natural history that occurs in adults with postrepair myelomeningocele and TCS? (3) What are the criteria for operative intervention? (4) What is the functional outcome for patients with postrepair myelomeningocele?

METHODS: A computerized search of the National Library of Medicine of the English-language literature published from 1966 to 2003 was performed. Articles pertaining to the clinical aspects and management of postrepair myelomeningocele in adults, TCS in adults resulting from closed defects, and pediatric patients with postrepair myelomeningocele were reviewed. No Level 1 or 2 data were located; however, the search yielded Level 3 and 4 evidence in the literature in which the clinical syndrome, underlying pathology, and the imaging and electrophysiological evaluation for TCS in adults are discussed.

CONCLUSIONS: Analysis of the available data indicates the following. (1) A lower lesion level predisposes patients to symptomatic tethering; moreover, orthopedic and urological deterioration will occur in the majority of these patients. (2) Tethered cord release should be considered for adult patients with postrepair myelomeningocele when clinical symptoms, imaging studies, urodynamics, and somatosensory evoked potentials are consistent with TCS. (3) Prompt, aggressive untethering surgery within 5 years of symptom onset, along with long-term follow up to check for delayed retethering, is recommended. The overall outcome for patients with postrepair myelomeningocele may not be as good as the outcome for adults with closed dysraphism.

PMID: 16156223