Epileptic spasms in paediatric post‐traumatic epilepsy at a tertiary referral centre

Aim. To recognize epileptic spasms (ES) as a seizure type after traumatic brain injury (TBI), accidental or non‐accidental, in infants and children. In the process, we aim to gain some insight into the mechanisms of epileptogenesis in ES. Methods. A retrospective electronic chart review was performed at the Children's Hospital of Michigan from 2002 to 2012. Electronic charts of 321 patients were reviewed for evidence of post‐traumatic epilepsy. Various clinical variables were collected including age at TBI, mechanism of trauma, severity of brain injury, electroencephalography/neuroimaging data, and seizure semiology. Results. Six (12.8%) of the 47 patients diagnosed with post‐traumatic epilepsy (PTE) had ES. Epileptic spasms occurred between two months to two years after TBI. All patients with ES had multiple irritative zones, manifesting as multifocal epileptiform discharges, unilateral or bilateral. Cognitive delay and epileptic encephalopathy were seen in all six patients, five of whom were free of spasms after treatment with vigabatrin or adrenocorticotropic hormone. Conclusion. The risk of PTE is 47/321(14.6%) and the specific risk of ES after TBI is 6/321 (1.8%). The risk of ES appears to be high if the age at which severe TBI occurred was during infancy. Non‐accidental head trauma is a risk factor of epileptic spasms. While posttraumatic epilepsy (not ES) may start 10 years after the head injury, ES starts within two years, according to our small cohort. The pathophysiology of ES is unknown, however, our data support a combination of previously proposed models in which the primary dysfunction is a focal or diffuse cortical abnormality, coupled with its abnormal interaction with the subcortical structures and brainstem at a critical maturation stage.     

The impact of COVID-19 on neurosurgical head trauma referrals and admission at a tertiary neurosurgical centre

BackgroundCOVID-19 has greatly impacted surgical specialities throughout the globe leading to a decrease in hospital admissions and referrals. Neurosurgery has seen a great decline in cases including head trauma leading to a negative impact on the development of neurosurgical trainees. The main objective of this study is to the identify changes in neurosurgical referrals, admissions and management during the COVID-19 pandemic. We also aim to assess how current practise could be adapted to help manage future pandemic peaks.MethodsData was collected for the first 31 days of lockdown during 2020 (23rd March – 22nd April) and compared to the same time period in the years 2016–2019. We assessed the number of referrals, admissions and clinical information of patients during this period with a key emphasis on head trauma.ResultsNeurosurgical head injury referrals and admissions reduced by 57.5% and 48.3% respectively during the first 31 days of lockdown when compared to the mean figures for the same period in the previous 4 years. This was also seen with head trauma with a 21.9% decline in referrals and 39.1% reduction in admissions for the period of interest. A significant decrease in length of stay (P < 0.001) was seen between 2020 and the years 2017–19.ConclusionThe impact of COVID-19 makes it imperative that we plan for future pandemics to lessen the impact on neurosurgery. Special considerations need to be taken so that trainees are sufficiently prepared for completion of training whilst still priotising patient safety and providing high quality care.     

Paediatric germ cell tumours of the central nervous system: Results and experience from a tertiary-referral paediatric institution in Australia

A retrospective analysis was conducted on consecutive patients with intracranial germ cell tumours diagnosed and treated from 1 January 1997 to 31 December 2007 to assess and determine demographic factors and treatment outcomes of children with these tumours treated in a major paediatric referral hospital in Australia. In this study, intracranial germ cell tumours represented 4.8% of paediatric brain tumours seen. Of the 21 patients identified, 15 (71.4%) were diagnosed with pure germinoma and six (28.6%) with non-germinomatous germ cell tumours (NGGCT) or mixed tumours. One patient received chemotherapy alone, two patients were treated with radiation alone and the remaining 18 received a combination of chemotherapy and radiotherapy. A total of 33 neurosurgical operations were performed with 15 biopsies via open, endoscopic or transphenoidal means; nine open resections; and nine procedures for hydrocephalus comprising seven third ventriculostomies and two ventriculoperitoneal shunts. For patients with pure germinomas, the 5-year disease-free rate (DFS) was 93.3%, and overall survival (OS) rate was 100% compared to NGGCT or mixed tumours (DFS 50%; OS 50%) (DFS p = 0.019, OS p = 0.004). The data presented show that pure germinomas carry a favourable prognosis. The data also support that treatment with induction chemotherapy followed by dose-attenuated radiotherapy is an effective alternative with results comparable to historical controls treated with craniospinal irradiation. Although chemoradiotherapy has become the mainstay of treatment in intracranial germ cell tumours, surgery remains integral to the management of this condition. Surgery remains important in establishing the histological diagnosis, as well as in the treatment of hydrocephalus. Furthermore, debulking procedures may be advocated in NGGCT as they are often resistant to chemotherapy.     

Outcome of "unknown" head injury patients at a tertiary care neurosurgical centre

CONTEXT: A large number of patients are admitted to hospitals in large cities without any identification. These "unknown" patients represent a unique problem in developing countries. There is no systematic study in world literature on this subgroup of patients. AIMS: To elucidate the natural history and outcome of traumatic brain injury patients admitted in the department of neurosurgery as "unknowns". SETTINGS AND DESIGN: Retrospective analysis of all traumatic brain injury patients admitted to the department of neurosurgery as "unknown", between January 2002 and March 2005. RESULTS: Three hundred and twenty five patients were admitted as unknowns over a 3 year and 3 months period. Most of the patients were young males and became known during their hospital stay. Overall, 33 patients stayed for longer than one month, with 4 of them staying for longer than 6 months. Mortality in mild, moderate and severe head injury group was 1%, 6% and 46% respectively. CONCLUSIONS: Unknown patients represent a unique subgroup in metropolitan cities like Delhi. Managing them raises several medico-legal issues. Many of them occupy hospital beds for longer duration than required. There is an acute shortage of rehabilitation homes in Delhi for long term care and rehabilitation of such patients.     

Screening for osteoporosis: a survey of older psychiatric inpatients at a tertiary referral centre

BACKGROUND: Osteoporosis causes much morbidity and mortality among elderly people. Older inpatients with severe mental illness may be at particular risk of osteoporosis because of factors including immobility, vitamin D deficiency and psychotropic medication. METHOD: We screened predominantly older inpatients with severe mental illness for osteoporosis using a peripheral DEXA scanner. Associations were examined between patient variables and osteoporosis as detected by DEXA scan, Z-scores and history of fragility fractures. Patients were followed up to see how many subsequently received drug treatment for osteoporosis. Patient acceptability of the scanning technique was also assessed. RESULTS: It was possible to scan 72/89 (81%) of patients. Mean T-score was -2.79 (SD 1.50) and mean Z-score -0.76 (SD 1.34). Forty-two (58%) patients had osteoporosis and 23 (32%) osteopenia. Twelve (17%) patients with a history of fragility fractures had osteoporosis on DEXA scan. A scan suggestive of osteoporosis and a history of fragility fractures were both associated with age of 75 years and over and thoracic kyphosis. Low Z-scores were associated with longer hospital stay and osteoporosis-predisposing drugs. Patient acceptability of scanning was high. At follow-up, only 7/16 (44%) patients were suitable for central scanning and 9/40 (23%) for bisphosphonates. CONCLUSION: Older institutional patients with severe mental illness are likely to be at high risk of developing osteoporosis. Peripheral DEXA scanning is acceptable to these patients and identifies those at high risk of fractures. Some behaviorally disturbed patients are not suitable for central scans or for some osteoporosis drug treatments.     

A clinicopathological and neuroradiological study of paediatric meningioma from a single centre

Paediatric meningiomas are rare intracranial neoplasms representing 0.4% to 4.1% of tumours in paediatric patients and 1.5% to 1.8% of all intracranial meningiomas. The goal of this study was to determine the epidemiology, clinical presentation, radiological features, morphological spectrum and treatment outcome of paediatric meningiomas. All evaluable reports of meningioma in children from 1 January 1999 to 31 December 2009 were retrieved from the database of our Department of Pathology and were assessed retrospectively. This study describes 12 patients (nine males, three females; age range, 4-18 years; mean, 12.8 years). Their age and sex distribution, presenting symptoms, neurological findings, location, neuroradiological and histopathological findings were reviewed and the results were compared with published reports. Atypical and malignant meningiomas seem to be more common in childhood and adolescence than in adulthood.     

Ten-year review of extradural spinal abscesses in a New Zealand tertiary referral centre

Despite being a well-understood pathology, patients with an extradural spinal abscess (ESA) still experience delays in diagnosis, with associated morbidity and mortality. This 10-year retrospective audit aimed to define the presentation, findings and prognosis of ESA in a New Zealand tertiary referral centre. Forty-two patients were diagnosed and treated between 1 May 1997 and 30 June 2007. The mean age was 55 years, with a male predominance of 1.6:1. Staphylococcus aureus was identified in 67% of patients. On presentation, 27/38 patients with back pain were afebrile and 2/37 patients had a normal C-reactive protein (CRP) level in the first 24 hours. Ten patients were discharged with neurological dysfunction and one died. One reason for the delays in diagnosis and appropriate referral could be the lack of “red flag” symptoms at the time of presentation. Attention needs to be paid to the history and nature of a patient’s back pain. We recommend routine CRP and full blood count to help raise clinical suspicion.     

Incidence of sudden unexpected death in an adult outpatient cohort with epilepsy at a tertiary referral centre.

Overall mortality, incidence of sudden unexpected death, and cause of death were determined in 601 adult outpatients with epilepsy at a tertiary referral centre. The patients were followed up from 1990 to 30 June 1993. There were 24 deaths among the 601 patients (1849 patient years) with a standardised mortality ratio of 5.1 (95% confidence interval 3.3-7.6) of which 14 were related to epilepsy. Underlying disease of which epilepsy was a symptom accounted for four deaths only. An incidence of sudden deaths (including seizure related) was of the order of 1:200/year. In conclusion, excess mortality in chronic epilepsy is more likely to be related to the epilepsy itself than to underlying pathology. The relatively high incidence of sudden deaths found in this hospital based cohort has important implications for patient management.     

The diagnosis of blocked cerebrospinal fluid shunts: a prospective study of referral to a paediatric neurosurgical unit

A prospective study was undertaken of all children referred to the Hospital for Sick Children with a provisional diagnosis of shunt blockage over a 5-month period. Fifty-two admissions were recorded, relating to 45 children, 5 of whom had multiple admissions. Only 19 of the 52 admissions led to a final diagnosis of shunt malfunction. No source of referral, whether by the child's general practitioner or from another hospital, was found to be more accurate than direct referral by the parents to the neurosurgical ward. Headache, vomiting and irritability were not significant indicators as to whether the child's shunt was actually blocked, and nor was the duration of the symptoms. Drowsiness was a significant, but not definite, indicator of shunt blockage, while pyrexia made it more likely that the patient had an alternative diagnosis. In 35 of the admissions a computed tomographic scan was performed: a normal scan, unchanged from previous scans, did not reliably exclude the diagnosis of shunt blockage. Percutaneous manometry via the reservoir of the shunt system was performed during 26 admissions: this investigation produced no false positives nor false negatives, but was equivocal in 5 cases, all of which were found at surgery to have a definite shunt blockage. The accuracy of the diagnosis of shunt blockage made prior to referral to a neurosurgical unit is discussed, together with the implications for resource use.     

Epidemiology of paediatric central nervous system tumours in Queensland,Australia

Within Australia, there is little epidemiologic information regarding paediatric central nervous system (CNS) tumours. This study examined the epidemiology of childhood CNS tumours at Queensland Children’s Hospital (QCH), the major paediatric referral centre for Queensland and northern New South Wales. We assessed the data from 221 newly diagnosed childhood CNS tumours across a five-year period from 2015 to 2019. Recurrent tumours were excluded. Data was collected on patient age, gender, histopathological diagnosis, tumour grade, anatomical site, and residential geographical location. The incidence within this period ranged from 2.65 to 3.85 cases per 100,000 children. The median symptom interval was 30 days (IQR 14–122) with presenting features similar to previous studies. The symptom interval was shorter for children zero to three years compared to children four years or older. The most frequent tumour was pilocytic astrocytoma, followed by medulloblastoma and Langerhans cell histiocytosis. The posterior fossa was the most frequent anatomical location for tumours. Our study demonstrated a shorter symptom interval in comparison to previous literature. The study was able to determine the incidence and presenting features within an Australian population.     

A genetic basis is identified in 74% cases of paediatric hyperCKaemia without weakness presenting to a tertiary paediatric neuromuscular centre

Paediatric hyperCKaemia without weakness presents a clinical conundrum. Invasive investigations with low diagnostic yields, including muscle biopsy, may be considered unjustifiable. Improved access to genome-wide genetic testing has shifted first-line investigations towards genetic studies in neuromuscular disease. This research aims to provide an evidence-based diagnostic approach to paediatric hyperCKaemia without weakness, a current gap in the literature. We identified 47 individuals (10-months to 16-years-old; 34 males, 13 females) from 43 families presenting with hyperCKaemia on two or more occasions, without weakness, from The Children's Hospital at Westmead Neuromuscular Clinic Database. Clinical features, investigations and outcomes were analysed via retrospective chart review. Genetic testing has been performed in 34/43. Genetic variants explaining hyperCKaemia were identified in 25/34 (74%) using multiplex ligation-dependent probe amplification, massive parallel sequencing, single gene testing and exome sequencing. Pathogenic/likely pathogenic variants were identified in 19 neuromuscular disease genes and six metabolic myopathy genes. Individuals with metabolic diagnoses had higher peak creatine kinase levels that sometimes normalized. Conversely, creatine kinase levels remained persistently elevated those with neuromuscular diagnoses. In summary, a genetic cause is found in most paediatric patients with hyperCKaemia without weakness informing clinical management and counselling. Thus, we propose a diagnostic algorithm for this cohort.     

Relationship of akathisia to aggressive and self-injurious behaviour: A prevalence study in a UK tertiary referral centre

INTRODUCTION: Akathisia is a common and distressing side-effect associated with antipsychotic drug administration. The relationship between akathisia and five forms of expressed aggression is investigated. METHOD: Sixty-four mentally disordered patients were assessed for the point prevalence of akathisia, using Barnes' rating scale for drug-induced akathisia. 1 The five types of aggression studied were: verbal abuse/ aggression, threatening behaviour/violence, physical aggression, destruction of property and deliberate self-harm, all of which are routinely recorded for patients. RESULTS: Fourteen subjects (21.9%) experienced akathisia, which was approximately four times more likely to occur in women than in men: four (6.3%) had pseudoakathisia. Akathisia was statistically significantly associated with threatening behaviour (P<0.05) and physical aggression (P<0.05). CONCLUSION: The data provide evidence for a relationship between the experience of akathisia and the expression of two forms of aberrant behaviour.     

Prevalence and diagnostic challenge of dystonia in Thailand: a service-based study in a tertiary university referral centre

Although the subspeciality of movement disorders was established in neurology more than 20 years ago, it is relatively new in Thailand, and while most physicians are generally aware of Parkinson's disease, they often are not familiar with dystonia. As one of the common movement disorders seen in general practice, a number of family and population studies have suggested that as many as two-thirds of patients with dystonia may be underdiagnosed and it is likely that misdiagnosis occurs frequently. Moreover, there is little information on the prevalence of dystonia in Thailand. The purpose of this study was to determine the prevalence and clinical profile of dystonia among Thai patients who came from the southern part of Bangkok, which is in the catchment area of Chulalongkorn University Hospital. In addition, the diagnostic accuracy of dystonia among referred patients was assessed. The medical records of 207 patients were reviewed and it was determined that a large proportion of them (71.9%) had focal dystonia with cervical dystonia being the most common form. Primary dystonia (68.1%) accounted for the majority of the cases. The prevalence of all forms of dystonia, primary dystonia and focal dystonia was 19.9, 13.6 and 14.3 per 100,000 persons, respectively. The diagnostic accuracy of dystonia among referred patients was 85.5%. The most common misdiagnosis was cervical spondylosis, followed by myofascial pain syndrome. Most patients had an average disease duration of 4 years before dystonia was finally diagnosed. Most patients with focal dystonia responded well to botulinum toxin therapy, with 13.3% suffering only mild transient adverse events. In spite of the limitations of this study, this data will initiate a process of increasing both patient and professional awareness of dystonia in Thailand.     

Cervical dystonia: Improved treatment response to botulinum toxin after referral to a tertiary centre and the use of polymyography

RationaleCervical dystonia is the most common form of (primary) dystonia. The first line of treatment for cervical dystonia is intramuscular injections with botulinum toxin. To optimise the response to botulinum toxin proper muscles selection is required. Pre-treatment polymyographic EMG in addition to clinical evaluation is hypothesised to be a good tool to improve muscle selection and treatment outcome.ObjectiveTo determine the efficacy of botulinum toxin treatment after adjacent polymyographic EMG in cervical dystonia patients referred to our tertiary referral centre with an unsatisfactory response to botulinum toxin treatment elsewhere.MethodsWe performed a retrospective analysis of 40 consecutive second opinion cervical dystonia patients. Standard polymyographic EMG was performed before treatment. We retrieved the Tsui scores and subjective evaluations from the first visit, after 12 weeks and after one year of treatment. In addition, we assessed the final outcome of treatment in our centre based on the records and asked the patients for their personal opinion about the effect of referral to our centre on their treatment response.ResultsAfter one year of treatment there was a significant improvement on both the Tsui scores (p < 0.01) and the subjective treatment evaluation (p < 0.001.) On their last visit 60% of the patients still continued treatment with a reasonable to good response.ConclusionA substantial amount of CD patients with an unsatisfactory response to botulinum toxin improved after polymyography and subsequent treatment with botulinum toxin in a tertiary referral centre.     

Non-traumatic brachial plexopathies, clinical, radiological and neurophysiological findings from a tertiary centre

OBJECTIVE: To establish the clinical characteristics, aetiology, neuro-physiological characteristics, imaging findings and other investigations in a cohort of patients with non-traumatic brachial plexopathy (BP). METHODS: A 3-year retrospective study of patients with non-traumatic BP identified by electromyography (EMG) and nerve conduction studies (NCS). Clinical information was retrieved from patients' medical charts. RESULTS: Twenty-five patients were identified. Causes of BP included neuralgic amyotrophy (NA) (48%), neoplastic (16%), radiation (8%), post infectious (12%), obstetric (4%), rucksack injury (4%), thoracic outlet syndrome (4%) and iatrogenic (4%). Patients with NA presented acutely in 50%. The onset was subacute in all others. Outcome was better for patients with NA. All patients with neoplastic disease had a previous history of cancer. MRI was abnormal in 3/16 patients (18.8%). PET scanning diagnosed metastatic plexopathy in two cases. CONCLUSIONS: NA was the most common cause of BP in our cohort and was associated with a more favourable outcome. The authors note potentially discriminating clinical characteristics in our population that aid in the assessment of patients with brachial plexopathies. We advise NCS and EMG be performed in all patients with suspected plexopathy. Imaging studies are useful in selected patients.     

The changing epidemiology of paediatric brain tumours: a review from the Hospital for Sick Children

Purpose  This study examines the changing epidemiology of paediatric brain tumours over the past three decades (1980–2008) in a single institution, SickKids, Toronto, Canada. Methods  We classified 1,866 surgical pathology cases of brain tumours in children under the age of 19 according to the World Health Organization 2007 consensus and analysed them by gender, histological tumour type, age distribution and decade. Results  Males showed a slightly higher predominance with 56.8% of cases overall. The main histological tumour types were low-grade (I/II) astrocytomas (26.4%), medulloblastoma (10.6%), anaplastic astrocytoma/glioblastoma multiforme (7.1%) and ependymoma (7.0%). Over three decades, an increasing proportion of certain tumour types, including pilocytic astroctoma, atypical teratoma/rhabdoid tumours and neuronal/mixed neuronal-glial tumours was seen. Conclusions  Our results are consistent with those published with similar methodologies in other countries. Any changes in the epidemiology of childhood central nervous system tumours over the past three decades may be attributed in part to changing classification systems, improved imaging technologies and developments in epilepsy surgery; however, continued surveillance remains important.     

A quarter of a century of neurosurgery: the value of a relational database to document trends in neurosurgical practice of a tertiary referral hospital.

OBJECTIVE AND IMPORTANCE: Increasing interest in evidence-based medicine has created a demand for accurate and accessible information on activity and trends in clinical practice. A database of all neurosurgery admissions at a teaching hospital maintained by a scientist has been utilised to examine changes in practice and complications from 1977 to 2001.METHODS: A relational database, set up in 1982, now contains an unbroken record of all neurosurgical admissions at Royal North Shore Hospital (RNSH) since 1976. It supplies information for morbidity and mortality meetings, research and administrative purposes. A total of 23,766 admissions from 1977 to 2001 were examined. Statistical analysis of trends in age, gender, length of stay (LOS), diagnostic mix, surgery rates and complications in admissions was based on diagnostic groupings. RESULTS: Proportions of vascular admissions rose and of trauma admissions fell. Mean age increased significantly for tumour, trauma and spinal patients; geometric mean LOS declined significantly for tumour, spine, vascular, cranial nerve and peripheral nerve groups. Concurrently, inpatient death rate fell significantly for tumour and vascular patients. Deep vein thrombosis (DVT) rose significantly for trauma, vascular, tumour, spinal and infection patients; pulmonary embolism (PE) rose significantly for tumour, trauma and spinal patients. There was no significant change in wound infection rate at approximately 3.5% of all operated patients. Wound haematoma rates fell significantly from 4.0% to 2.9% while the rate of postoperative cerebrospinal fluid (CSF) leak rose significantly from 0.5% to 2.0% of all operated patients. CONCLUSION: The value of the database is demonstrated by its ability to provide analysis which shows statistically significant changes over time. Declining death rate and LOS indicate improved efficiency in managing patients, but these are offset by rising rates of CSF leak, DVT and PE. Such rises reflect the changing patterns of casemix and surgery performed, and increasing financial pressures on hospital departments.     

Management of refractory status epilepticus at a tertiary care centre in a developing country

BackgroundRefractory status epilepticus (RSE) is a common Neurological Emergency with increased mortality and morbidity in developing countries where facilities of intubation, adequate ventilation, Intensive Care Units (ICUs) and general anaesthesia are not ubiquitously available. Treatment protocols use antiepileptic drugs (AEDs) and need ICU facilities after failure of standard AEDs. Our aim was to see the response to two additional drugs in the armamentarium against refractory status, that is, valproate and levetiracetam.MethodsPatients with generalized RSE admitted in neurology and neurosurgery services at AIIMS during December 2006 to June 2008 were included in the study. The patients were allotted to two groups based on certain criteria. Demographic details, reason for delay, etiology precipitating status, ongoing AEDs therapy, duration of status, the time taken for cessation along with clinical, EEG and MRI correlates were noted. Outcome parameters were analyzed by an independent blinded observer.Results82 patients with RSE were studied out of which 41 patients were given IV valproate (Group A) and 41 patients were given IV levetiracetam (Group B). Cessation of status failed in 13 patients in valproate group and 11 patients in levetiracetam group. Majority of the patients did not require ICU settings despite being classified as refractory.ConclusionRSE can be controlled with intravenous loading and maintenance of valproate or levetiracetam which do not cause respiratory depression, hypotension, need of intubation and ICU care. These must always be considered in a developing country scenario where ICU facilities are not always available or while transporting to centres where these facilities are available.