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Biomedical subjects

M Steinlin

Publications and source records attributed to M Steinlin.

At least 19 recordsLinked to original sources
PubMed2007

Randomised controlled trials of homeopathy in hyperactive children: treatment procedure leads to an unconventional study design. Experience with open-label homeopathic treatment preceding the Swiss ADHD placebo controlled, randomised, double-blind, cross-over trial.

BACKGROUND: Treatment of patients with attention deficit hyperactivity disorder (ADHD) with homeopathy is difficult. The Swiss randomised, placebo controlled, cross-over trial in ADHD patients (Swiss ADHD trial) was designed with an open-label screening phase prior to the randomised controlled phase. During the screening phase, the response of each child to successive homeopathic medications was observed until the optimal medication was identified. Only children who reached a predefined level of improvement participated in the randomised, cross-over phase. Although the randomised phase revealed a significant beneficial effect of homeopathy, the cross-over caused a strong carryover effect diminishing the apparent difference between placebo and verum treatment. METHODS: This retrospective analysis explores the screening phase data with respect to the risk of failure to demonstrate a specific effect of a randomised controlled trial (RCT) with randomisation at the start of the treatment. RESULTS: During the screening phase, 84% (70/83) of the children responded to treatment and reached eligibility for the randomised trial after a median time of 5 months (range 1-18), with a median of 3 different medications (range 1-9). Thirteen children (16%) did not reach eligibility. Five months after treatment start, the difference in Conners Global Index (CGI) rating between responders and non-responders became highly significant (p = 0.0006). Improvement in CGI was much greater following the identification of the optimal medication than in the preceding suboptimal treatment period (p < 0.0001). CONCLUSIONS: Because of the necessity of identifying an optimal medication before response to treatment can be expected, randomisation at the start of treatment in an RCT of homeopathy in ADHD children has a high risk of failure to demonstrate a specific treatment effect, if the observation time is shorter than 12 months.

Adolescent↗
PubMed2006

Neuropsychological problems after paediatric stroke: two year follow-up of Swiss children.

AIM: The aim of this study was to obtain information about neurological and cognitive outcome for a population-based group of children after paediatric ischaemic stroke. METHODS: Data from the Swiss neuropaediatric stroke registry (SNPSR), from 1.1.2000 to 1.7.2002, including children (AIS 1) and neonates (AIS 2). At 18-24 months after a stroke, a follow-up examination was performed including a history, neurological and neuropsychological assessment. RESULTS: 33/48 children (22 AIS 1, 11 AIS 2) participated in the study. Neurological outcome was good in 16/33. After childhood stroke mean IQ levels were normal (94), but 6 children had IQ < 85 (50-82) and neuropsychological problems were present in 75%. Performance IQ (93) was reduced compared to verbal IQ (101, p = 0.121) due to problems in the domain of processing speed (89.5); auditory short-term memory was especially affected. Effects on school career were common. Outcome was worse in children after right-sided infarction. Children suffering from stroke in mid-childhood had the best prognosis. There was no clear relationship between outcome and localisation of the lesion. After neonatal stroke 7/11 children showed normal development and epilepsy indicated a worse prognosis in the remaining 4. CONCLUSION: After paediatric stroke neuropsychological problems are present in about 75% of children. Younger age at stroke as well as an emergence of epilepsy were predictors for worse prognosis.

Adolescent↗
PubMed2005

[Neuropediatric emergencies--epileptic seizures and acute hemiplegia].

Status epilepticus and stroke are life-threatening neurological emergencies and immediate recognition and medical management are imperativ. There is a serious risk of neurological sequelae. To limit secondary brain damage a prolonged seizure has to be treated without delay. After a short history and clinical evaluation (principles of resuscitation) a benzodiazepine (usually diazepam or lorazepam) has to be administered and in case of persistence of seizure-activity phenytoin or phenobarbital. Blood- and urine-sample must be collected in the acute phase to perform further metabolic or toxic examinations. A paralysis with acute onset is suspicious for ischaemic or haemorrhagic stroke. A precise neurological examination is mandatory for accurate neuroradiological work-up and to initiate appropriate investigations for risk factors. These patients require an immediate interdisciplinary treatment in a tertiary care centre with neuropaediatry, neuroradiology and neurosurgery.

Acute Disease↗
PubMed2005

The first three years of the Swiss Neuropaediatric Stroke Registry (SNPSR): a population-based study of incidence, symptoms and risk factors.

We report the results of three years of the population-based, prospective Swiss NeuroPaediatric Stroke Registry (SNPSR) of children (up to 16 years) with childhood arterial ischaemic stroke (AIS1), neonatal stroke (AIS2), or symptomatic sinus venous thrombosis (SVT). Data on risk factors (RF), presentation, diagnostic work-up, localisation, and short-term neurological outcome were collected. 80 children (54 males) have been included, 40 AIS1, 23 AIS2, and 17 SVT. The data presented will be concentrated on AIS. The presentation for AIS1 was hemiparesis in 77% and cerebellar symptoms and seizures in 20%, respectively. AIS2 presented in 83% with seizures and in 38% with abnormality of muscle tone. Two or more RF were detected in 54%, one RF in 35%. The most prominent RF for AIS1 were infections (40%), followed by cardiopathies and coagulopathies (25% each). AIS2 were frequently related to birth problems. Neurological outcomes in AIS1 and AIS2 were moderate/severe in 45 % and 32 %, respectively. The outcome correlated significantly with the size of infarction (p = 0.013) and age at stroke (p = 0.027). The overall mortality was 6%. Paediatric stroke is a multiple risk problem, which leads to important long-term sequelae.

Adolescent↗
PubMed2003

Isotropic apparent diffusion coefficient mapping of postnatal cerebral development.

Diffusion-weighted imaging (DWI) allows us to image the motion of tissue water. This has been used to demonstrate acute ischaemia. Diffusion imaging is also sensitive to water movement along neuronal tracts. Our objective was to map brain maturation in vivo using maps of apparent diffusion coefficient (ADC). We studied 22 children without neurological disease aged between 2 and 720 days. MRI was performed at 1.5 tesla. Multislice single-shot echoplanar DWI was performed at b 0 and 1000 s/mm(2). ADC maps were generated automatically and measurements were performed in the basal ganglia, frontal and temporal white matter and the pons. There was a decrease over time in water diffusion in the areas examined, most marked in the frontal (0.887-1.898 x 10(-3) mm(2)/s) and temporal (1.077-1.748 x 10(-3) mm(2)/s)lobes. There was little change, after an initial decrease, in the basal ganglia (0.690-1.336 x 10(-3) mm(2)/s). There was a difference in water diffusion between the anterior (0.687-1.581 x 10(-3) mm(2)/s) and posterior (0.533-1.393 x 10(-3) mm(2)/s) pons. These changes correlate well with those observed in progressive myelination: the increased water content probably reflects incomplete myelination and the decrease with time in water motion reflects the increase in myelinated brain.

Brain Mapping↗
PubMed2003

[Suspected muscular disease: what to do?].

During the last decades important progress in knowledge of hereditary neuromuscular problems could be achieved. Unfortunately, therapeutic management has not yet improved significantly, but diagnostic investigations have become less invasive for most disorders. For the clinician, the most important problem is still realizing that the complains of the patient could be a symptom of a neuromuscular problem. This should be followed by a careful history and clinical examination, to achieve a topical diagnosis (from motoneuron to muscle) and if ever possible already a clinical suspicion of the diagnosis. Investigations like neurophysiological technics, lab investigations, forearm ischemic exercise test or even muscular biopsy might be helpful to achieve this goal. Nowadays verification of the diagnosis is done in most cases by genetic DNA testing or by specific immunohistochemical staining or enzyme determination in muscular biopsy. This diagnostic way enhances the importance of history and clinical examination even in today's modern neurology.

Biopsy↗
PubMed2003

Acute leukoencephalopathy after inhalation of a single dose of heroin.

We describe extended and repeat magnetic resonance (MR) examinations in the case of a 16-year-old male who developed acute left-sided sensorimotor hemiplegia after a single dose of inhaled heroin. MRI revealed symmetrical hyperintense signals in T 2 -weighted images and massive diffusion disorders in the diffusion weighted images predominantly in parieto-occipital subcortical white matter and both ventral globi pallidi with preservation of U fibers and no brain oedema. MR spectroscopy data were compatible with combined hypoxic and mitochondrial damage resulting in axonal injury without demyelination. Normal values and variations had been obtained from spectra of five age-matched subjects. This is the first reported MR follow-up study of leukoencephalopathy occurring acutely after a first inhaled dose of heroin. We postulate that toxic spongiform leukoencephalopathy in heroin addicts may be the outcome of a complex mechanism directly triggered by heroin and causing mitochondrial as well as hypoxic injury in specific and limited areas of white matter.

Acute Disease↗
PubMed1999

Cognitive impairments in patients with congenital nonprogressive cerebellar ataxia.

OBJECTIVE: To report neuropsychologic functions and developmental problems of patients with congenital nonprogressive cerebellar ataxia. BACKGROUND: Growing interest in cerebellar function has prompted closer attention to cognitive impairments in patients with cerebellar damage. METHODS: The authors studied 11 patients with nonprogressive congenital ataxia (NPCA) with Wechsler's intelligence testing, with additional tests of attention, memory, language, visual perception, and frontal functions. RESULTS: Seven of the 11 patients had an IQ of 60 to 92, with marked nonverbal deficits and subnormal to normal verbal performance (group A). Four patients had an IQ of 30 to 49 without pronounced profile asymmetry (group B). Four of the 7 group A patients had decreased alertness and sustained attention, but all had normal selective attention. Tests of frontal functions and memory yielded higher verbal scores than nonverbal scores. There was no deficit on the Aachener Naming Test (similar to the Boston Naming Test), because there were marked difficulties in the majority with visuoconstructive tasks and visual perception. Group B was significantly abnormal in almost all subtests, having a less prominent but similar profile. CONCLUSION: Patients with NPCA have significant cognitive deficits with an asymmetric profile and better verbal than nonverbal performance. Effects on nonverbal performance of longstanding deficits in visuospatial input during learning, the influence of impaired procedural learning, and asymmetric plasticity of the cerebral hemispheres may contribute to this uneven neuropsychological profile.

Adolescent↗
PubMed1999

Neonatal Escherichia coli meningitis: spinal adhesions as a late complication.

We describe two boys who had severe spinal complications in adolescence after a favorable initial recovery from neonatal Escherichia coli meningitis. Due to spinal granulomatous adhesions, one boy died after an attempted scoliosis operation (high cord lesion). The other showed severe progressive neurological deterioration with spinal and cerebellar symptoms. Conclusion The severe complication of chronic arachnoiditis with spinal adhesion may occur many years after neonatal acute bacterial meningitis.

Adolescent↗
PubMed1999

Interhemispheric interactions analyzed by coherence during flexor spasms.

OBJECTIVE: We used coherence analysis to test for leading discharges on an ipsilateral right mesial temporal lesion in a 5 year old boy with flexor spasms. METHOD: Digital EEG analysis with video-EEG telemetry was performed preparatory to epilepsy surgery. RESULTS: Study of 10 spasms with head drop and subsequent flexion of both arms demonstrated an interhemispheric time lag with secondary bilateral synchrony, with a mean difference of 17 ms. The right hemisphere led. After a lesionectomy with resection of epileptic regions (performed with electrocorticographical guidance), the patient has been seizure-free for 4 years. Pathology confirmed a low-grade mixed glioma and cortical dysgenesis. CONCLUSION: The coherence analysis demonstrated a pathway of secondary generalization, confirming that the lesional side was leading during ictal generalized discharges in flexor spasms.

Brain↗
PubMed1998

Cerebellar involvement in metabolic disorders: a pattern-recognition approach.

Inborn errors of metabolism can affect the cerebellum during development, maturation and later during life. We have established criteria for pattern recognition of cerebellar abnormalities in metabolic disorders. The abnormalities can be divided into four major groups: cerebellar hypoplasia (CH), hyperplasia, cerebellar atrophy (CA), cerebellar white matter abnormalities (WMA) or swelling, and involvement of the dentate nuclei (DN) or cerebellar cortex. CH can be an isolated typical finding, as in adenylsuccinase deficiency, but is also occasionally seen in many other disorders. Differentiation from CH and CA is often difficult, as in carbohydrate deficient glycoprotein syndrome or 2-L-hydroxyglutaric acidaemia. In cases of atrophy the relationship of cerebellar to cerebral atrophy is important. WMA may be diffuse or patchy, frequently predominantly around the DN. Severe swelling of white matter is present during metabolic crisis in maple syrup urine disease. The DN can be affected by metabolite deposition, necrosis, calcification or demyelination. Involvement of cerebellar cortex is seen in infantile neuroaxonal dystrophy. Changes in DN and cerebellar cortex are rather typical and therefore most helpful; additional features should be sought as they are useful in narrowing down the differential diagnosis.

Adolescent↗
PubMed1998

Non-progressive congenital ataxias.

Congenital ataxias (CA) are rare, predominantly non-progressive syndromes characterized by marked hypotonia, developmental delay followed by the appearance of ataxia. Most children show marked speech and cognitive developmental problems. Non- progressive CA (NPCA) can be divided into pure CA without additional symptoms and syndromes with CA. Pure CA can be due to cerebellar malformations as (hereditary or non-hereditary) cerebellar hypoplasia, Dandy Walker syndrome, or occasionally supratentorial abnormalities. Ataxic syndromes are less frequent, but more distinctive. There are syndromes (e.g. Joubert syndrome) where ataxia is a cardinal feature and others where ataxia is only an occasional symptom. Acquired ataxias, due to congenital cytomegalovirus infection or perinatal problems, form a small third group. In about half of all cases with NPCA, aetiology and inheritance are still unknown. Diagnosis of NPCA is made by a typical history and careful clinical examination. Diagnosis of a more distinctive ataxic syndrome may be possible on clinical grounds. Neuroimaging with special attention to the posterior fossa will aid accurate clinical classification. Early progressive ataxias require careful differentiation from other types.

Ataxia↗
PubMed1998

Congenital ataxia of parietal origin? Report of two cases.

Congenital cerebellar ataxia is usually thought to be of cerebellar origin. We report two children with congenital cerebellar ataxia, in whom neuroimaging investigations suggest the possibility of a parietal etiology. The two boys showed hypotonia, delayed motor and cognitive development followed by marked, truncally pronounced ataxia. In one case infantile spasms were treated successfully with adrenocorticotropic hormone, although in follow-up the child suffered from occasional seizures. Magnetic resonance imaging showed in one case parieto-occipital pachygyria and in the other there was marked pachygyria, most pronounced over the parieto-occipital area. In both children cerebellar structures were normal. Cerebello-parietal connections are known to be responsible for acquired parietal limb ataxia. Although not proven, parietal lesions are the most likely etiology of congenital cerebellar ataxia in these two children. Therefore, cerebral, especially parietal pathology must be considered in children with congenital ataxia.

Brain Diseases↗
PubMed1998

Non-progressive congenital ataxia with or without cerebellar hypoplasia: a review of 34 subjects.

Information on the long-term development of larger series of children with non-progressive congenital ataxia (NPCA) is scarce. We have updated a personal cohort of subjects previously diagnosed as having NPCA. Children with brain malformations, acquired neurological illness, or defined syndromes were excluded. From 58 subjects, 34 were available for review (including three pairs of siblings). All our subjects had delayed motor and speech development. Truncal ataxia persisted but became less significant. Two subjects developed spasticity and three a focal dystonia. Epilepsy was a feature in 10 of the subjects. Cognitive impairment was present in 22 of 34 subjects. MRI was normal in 15 of 27. There were no obvious correlations between degree of motor delay, severity of ataxia, cognitive impairment, and neuroimaging. Although genetically and clinically not a homogeneous entity, NPCA is a helpful diagnostic label. Major problems arise in the majority of subjects related to cognitive impairment and less to neurological symptoms. Early individual prognosis is not possible from early developmental milestones, neurological signs, or neuroimaging.

Adolescent↗
PubMed1998

Adverse effects of vigabatrin in Angelman syndrome.

New antiepileptic drugs designed for enhancing GABAergic inhibition, such as vigabatrin (VGB) may be effective in Angelman syndrome (AS), because associated convulsions could be related to a reduced GABA-receptor density or receptor abnormality. From our preliminary experiences in four children with AS treated with VGB, we conclude that it may induce and increase seizures in patients with AS.

Adult↗