Living with chronic illness: a retrospective study of patients shunted for hydrocephalus and their families

Thirty-six patients who had had shunts for hydrocephalus between 14 and 36 years ago, and their parents and siblings were interviewed about the effects of chronic illness on their lives. Despite reports of severe financial, physical and emotional stress, most judged the quality of their family relationships and lives to be good. General outcome was poorest for those with unfavourable medical outcomes and/or questionable prospects for future independence. Almost all respondents were satisfied with the level and duration of treatment they had received.     

Long-term prognosis for children with shunted hydrocephalus

We reviewed the previous medical history and the social status of all patients of Oulu University Central Hospital who had had in the age range 16–26 years shunted hydrocephalus (HC) during childhood. Of 42 patients selected 7 had died and another 5 had been institutionalized for severe mental handicap. Shunts had been changed a total of 103 times in 29 patients still living. The most common reason for a reoperation was blockage. Half of the patients re-examined showed neurological abnormalities or epilepsy. Both the verbal and the nonverbal IQ of the patients remained weak to average. Even though the patients' medical prognosis was fair, their social maturation did not keep up with their physical abilities. One-third were receiving or had received vocational training, but only a few were working. Up to one-quarter of the patients with shunted HC were at home without any meaningful work activities.     

Epileptic encephalopathy with continuous spikes and waves during sleep in children with shunted hydrocephalus: a study of nine cases

Purpose: We present a series of nine patients with early‐onset hydrocephalus who had seizures and continuous spikes and waves during slow sleep (CSWS) associated with neurocognitive and motor deterioration. Methods: Six boys and three girls aged 9–16 years (mean 11.3 years) were studied. [Correction added after online publication 12‐Apr‐2008: Number of girls and boys has been updated.] All patients underwent clinical examinations, electroencephalographic evaluations, neuroradiological imaging and neuropsychological assessment at first examination. Antiepileptic drugs (AEDs) were given in all cases and changed according to clinical and EEG evolution. Results: Onset of epilepsy occurred from age 8 to 60 months (mean 19.6 months and median 14 months) with focal seizures with or without secondary generalized tonic–clonic seizures. Between ages 6 and 13 years (mean 10.4 years and median 8 years), hyperkinesia, aggressiveness, and poor socialization appeared in all nine cases. Reduced attention span, deterioration of language, and temporospatial disorientation were found in three of them. Negative myoclonus was found in two patients. The EEG showed CSWS. Response to change in treatment was good in all patients. None of the patients had relapses, seven of them have remained seizure free, and two continued having sporadic focal motor seizures during 2–5 years (mean 3 years) of follow‐up. Conclusion: In children with early‐onset hydrocephalus, particularly with behavioral and language disturbances and/or motor deterioration, CSWS should be considered. Periodic EEG recordings during sleep should be done in these children. The early identification of this particular electroclinical picture is crucial to start adequate treatment to avoid progressive cognitive deterioration.     

Pre- and postoperative cerebrospinal fluid absorption studies in patients with myelomeningocele shunted for hydrocephalus

Nine infants with myelomeningocele and hydrocephalus had cerebrospinal fluid formation and absorption studies before insertion of a ventriculoperitoneal shunt. Six were also tested postoperatively. The preshunt formation rate varied from 0.21-0.42 ml.min-1 and the cerebrospinal fluid absorption rate or CSF conductance (Ccsf) varied from 0.008 to 0.031 ml.min-1.mm Hg-1. The postoperative formation rate varied from 0.21 to 0.39 ml.min-1. The postoperative CSF conductance (Ct) was calculated as the sum of Ccsf and the shunt conductance (Cs). The relationship between the Ccsf and Cs curves was determined by matching the sum of these curves to the postoperative data points using the method of least-squares. The data suggests that the shunt may work directly or indirectly to establish a new resting pressure; the latter by stabilizing the sagittal sinus pressure which preoperatively increased concomitantly with the CSF pressure and thus enhanced an already-existing absorption deficit.     

Intelligence outcome in children with shunted hydrocephalus of different etiology

Children with shunted hydrocephalus of differing etiologies were assessed. There was a more selective loss of non-verbal than of verbal intelligence. The verbal and visuo-perceptual abilities were affected by different sets of variables. Variables related to the hydrocephalus had no effects, while associated supratentorial malformations significantly affected nonverbal functions.Presented at the Consensus Conference: Hydrocephalus '92, Assisi, Italy, 26–30 April 1992     

Eight-second MRI scan for evaluation of shunted hydrocephalus

Introduction

Pediatric patients harboring shunts placed early in life are subjected to numerous radiographic studies during development of their central nervous system. Radiation is detrimental to these young patients. MRI avoids the risk of radiation but is thought more difficult due to the increased time a young patient must lie motionless during scan acquisition. Optimal radiographic interrogation would be quick, radiation-free, and allow adequate ventricular evaluation.

Methods

We queried the electronic medical records system of the senior author (SE) for the terms “hydrocephalus” and “shunt malfunction.” All patients currently younger than 18?years were included. In the last 5?years, pediatric patients have been evaluated in an office setting with a limited MRI sequence (T1 sagittal, T2 axial, T1 axial, and DWI) lasting a total of 178?s. In the event of significant motion artifact, the total sequence is abandoned and an 8-s?T2 diffusion-weighted scan is performed.

Results

Forty-four patients were included in the study (20 males, average age 10.4?yrs). Eighty-eight rapid acquisition scans were obtained. Adequate ventricular evaluation was performed without sedation in every case. In each instance where there was motion, the 8-s scan provided adequate ventricular evaluation.

Conclusion

Rapid acquisition MRI scanning avoids the deleterious cumulative effects of radiation in pediatric patients and allows adequate evaluation of the ventricles without the need for sedation.     

Outcome of shunted posthemorrhagic hydrocephalus in premature infants

Patient histories of 29 infants were reviewed whose birth weights were less than 2,000 gm and who had received ventricular shunts in the neonatal period for posthemorrhagic hydrocephalus. This procedure was performed at a time when routine screening of low birth weight infants for intracranial hemorrhage was not undertaken and serial lumbar puncture usually was not employed. The overall outcome was poor, with 62% of shunted infants either dying or surviving with moderate or severe handicap. Neurodevelopmental outcome was associated with the interval between the diagnosis of hydrocephalus and shunting; an adverse outcome was associated with an increased interval. Current practices for treating posthemorrhagic hydrocephalus are discussed.     

Ventriculoperitoneal shunt as a primary neurosurgical procedure in newborn posthemorrhagic hydrocephalus: report of a series of 47 shunted patients

Purpose

Intraventricular hemorrhage is the most common cause of infantile acquired hydrocephalus. Our objective is to determine if the implantation of ventriculoperitoneal shunt in posthemorrhagic hydrocephalus as a primary and definitive neurosurgical treatment, with no previous temporary procedures, would decrease complication rates with good functional outcomes.

Methods

Two hundred seventy-one patients with germinal matrix hemorrhage were diagnosed at the Carlos Haya Hospital between 2003 and 2010. Forty-seven patients underwent ventriculoperitoneal shunt after developing symptomatic hydrocephalus. The minimum weight required for shunt implantation was 1,500 g. We recorded complications related to the surgical procedure and analyzed functional state with a self-developed four-grade scale.

Results

One hundred thirty-nine (51.3 %) patients with intraventricular hemorrhage developed ventricular dilatation, but only 47 patients (17.34 %) needed shunting. In seven cases, temporary neurosurgical procedures were performed, but in all of them, this was followed by ventriculoperitoneal shunt implantation. The infection rate was 4.25 %, and shunt obstruction rate was 4.25 %. More than 80 % of patients were classified as good or excellent functional state. Mean follow-up period was 38.75 months (SD, 27.09; range, 1–102 months).

Conclusions

Ventriculoperitoneal shunting as a primary neurosurgical treatment in posthemorrhagic hydrocephalus would decrease surgical morbidity with good functional outcome.     

Continuous spikes and waves during sleep in children with shunted hydrocephalus

Focal epileptiform abnormalities in awake children submitted to ventricular shunting are well described in the literature, but there are few reports about EEG patterns during sleep. We studied 20 children affected by hydrocephalus of various aetiology and submitted to shunting during the first year of life. We found focal abnormalities in all the children and in 95% of cases they were on the same side as the shunt; in 65% of cases they had an amplitude of 300 mV or more. During sleep there was activation of abnormalities in all subjects, and in 33% we found continuous spikes and waves during slow sleep (CSWS). We discuss the aetiology of CSWS and its possible role in causing the neuropsychological disturbances of our patients. Received: 19 May 1997 Revised: 19 December 1997     

Psychosocial difficulties in patients with episodic migraine: a cross-sectional study

To explore the relationships between psychosocial difficulties (PSDs), quality of life (QoL), and disability and to explore the degree to which PSDs can be predicted by demographic variables, clinical variables, and risk and protective factors. Patients with episodic migraine completed a protocol inclusive of PARADISE 24 questionnaire (the 24-item Psychosocial Difficulties Relevant to Brain Disorders questionnaire), a new questionnaire that captures PSDs relevant to brain disorders, and assessments of disability, QoL, disease severity, presence of comorbidities, social support, and clinical and risk factors (i.e., smoking and body mass index). Spearman’s correlation was used to address the relationship between PARADISE 24, and the assessments of disability and QoL; multivariable linear regression analysis was carried out to address PARADISE 24 predictors. Eighty patients were enrolled (86.3 % females, mean age 44.5). PARADISE 24 was well correlated with disability (ρ = 0.787) and moderately with QoL (ρ = ?0.526). The regression analysis shows that younger age, higher migraine frequency, higher comorbidities index and being a smoker were predictors of PARADISE 24 (R ²: 0.470). Addressing the burden associated with PSDs in migraineurs is important as these might be the reason why patients look for specialists in headache disorders. PARADISE 24 represents a viable way to address patients’ difficulties in daily practice.     

Gait disturbance in normal pressure hydrocephalus: a clinical study

The origin of gait disturbance in normal pressure hydrocephalus (NPH) is poorly understood. We assessed the frequency of frontal and sub-cortical hypokinetic gait clinical features and changes in gait variables after lumbar puncture (LP), in 15 patients with idiopathic NPH. Hypokinesia and disequilibrium were the main features, suggesting dysfunction of the basal ganglia (BG) and the frontal lobe (FL), respectively. Disequilibrium responded worse to LP and frontal signs were more frequent in patients that did not improve after LP. This could be related to earlier disturbance of frontal gait control.     

Death in shunted hydrocephalic children: a follow-up study

Introduction The authors previously conducted a retrospective study regarding deaths from CSF shunt failure to identify circumstances surrounding shunt malfunction-related deaths in children in the modern era. Materials and methods Using the same methodology, we conducted a follow-up study to determine whether recent policy and procedural changes instituted since the time of the first study had effected a change in the mortality rate of our shunted patient population. Results Thirty-nine original patient records (of patients seen at Children’s Hospital who died with the diagnosis of hydrocephalus between 1998 and 2004) were identified and reviewed for inclusion into the study. Only four (10.3%) were found to have died directly as a result of shunt malfunction. Conclusions Our rate of shunt malfunction death has decreased over time. It is reasonable to attribute part of this decline to continued improvements in diagnostic and therapeutic techniques and to the use of surveillance scans to identify asymptomatic patients with shunt failure. However, we believe the majority of this decline is due to two additional factors: (1) increased nursing staff and (2) effective patient/family education.     

MRI morphometric study and correlation with cognitive functions in young adults shunted for congenital hydrocephalus related to spina bifida

We studied the morphometric MRI findings and their correlation with cognitive functions in a population of 10 young adults shunted for congenital hydrocephalus related to spina bifida. Morphometric MRI analysis included measurement of the ventricular dilatation index, frontal and occipital parenchymal thickness and the size of the corpus callosum. The neuropsychological status was evaluated, notably to look for a discrepancy between verbal and performance skills, a finding which has previously been described in hydrocephalic children. We also investigated whether there was a correlation between cognitive function and cerebral morphometric indexes. In each case, MRI demonstrated the structural changes associated with the Chiari II malformation. The size of the lateral ventricles varied, ranging from important dilatation to small ventricles. Six patients had only partial development of the corpus callosum. All patients had a normal global IQ. In our population of young adults, we did not observe any discrepancy between verbal and visuospatial performances as has been described in children with hydrocephalus. We found no relationship between cognitive function and ventricle dilatation or parenchymal thickness or between the size of the corpus callosum and callosal transfer.     

神经内镜下和传统分流术治疗脑积水的疗效评价

目的 评价神经内镜下第三脑室底造瘘术(ETV)或联合神经内镜引导下脑室腹腔分流术(EVPS)与传统分流术治疗脑积水的临床效果和并发症.方法 深圳大学第一附属医院神经外科自2002年6月至2009年6月共手术治疗299例脑积水患者,其中神经内镜组(98例)患者行神经内镜下ETV或联合EVPS(20例梗阻性脑积水患者仅行ETV,78例行ETV联合EVPS),传统分流组(201例)患者行传统分流术(196例行脑室腹腔分流术,5例行脑室心房分流术),分析2组患者的临床资料并比较其临床疗效和并发症的发生率.结果 与传统分流组比较,神经内镜组远期疗效好、堵管发生率低、置管位置准确率高、置管次数少,差异均有统计学意义(P<0.05).结论 应用神经内镜治疗不同原因脑积水是有益的手术方法,具有远期疗效好、堵管发生率低、置管位置准确等优势.     

Memory tests in first-degree adult relatives of schizophrenic patients: a meta-analysis

BACKGROUND: Memory deficits have been clearly demonstrated in schizophrenic patients. However, studies of memory performances in their relatives compared to normal controls provide conflicting results. A meta-analysis was carried out to synthesize all the published data. Unlike previous meta-analyses, which were based on composite scores, we analyzed each memory test separately. This prevents theoretically questionable choices in grouping variables, leads to results with clearer implications for applied research (e.g. the best choice of a test according to its sensitivity) and is more productive in suggesting explanatory hypotheses. METHOD: We initially selected 77 potentially relevant articles, but only 19 met our inclusion criteria. These articles provided data on eight different tasks, from five different memory tests: four tests from the Wechsler Memory Scale (WMS) and the California Verbal Learning Test (CVLT). For each task, we assessed data homogeneity, identified the outliers if any and then estimated effect sizes and tested publication bias using funnel plots. RESULTS: Adult relatives of schizophrenic patients were significantly impaired on most, but not all, tasks. The largest deficits were observed for the verbal paired associates test, the logical stories the digit span forward test and the digit span backward test. We found no significant differences in tasks of delayed recall, when deficits in immediate conditions (reflecting encoding) were taken into account. CONCLUSIONS: Adult relatives of schizophrenic patients have wide but not severe memory impairments. The size of estimated effects suggests that encoding processes are impaired, whereas storage and retrieval processes are relatively unaffected.     

Three-year survival and functional outcome of patients with idiopathic adult hydrocephalus syndrome

The functional outcome of 42 patients with idiopathic adult hydrocephalus syndrome (IAHS) was followed over a 3-year period after shunting. Survival curves were compared with those of age-matched healthy elderly subjects and patients with first-ever ischemic stroke. Twenty-seven patients with IAHS were improved 3 months after the operation and 11 remained improved at the 3-year follow-up. The case fatality in patients with stroke and those with IAHS was similar (32% versus 28%), but the relative risk of death among IAHS patients compared to a general elderly population was 3.3.