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M Kieslich

Publications and source records attributed to M Kieslich.

At least 19 recordsLinked to original sources
PubMed2006

Proton magnetic resonance spectroscopy in childhood brainstem lesions.

BACKGROUND: Diagnosis of brainstem lesions in children based on magnetic resonance imaging alone is a challenging problem. Magnetic resonance spectroscopy (MRS) is a noninvasive technique for spatial characterization of biochemical markers in tissues and gives information regarding cell membrane proliferation, neuronal damage, and energy metabolism. METHODS: We measured the concentrations of biochemical markers in five children with brainstem lesions and evaluated their potential diagnostic significance. Images and spectra were acquired on a 1.5-T imager. The concentrations of N-acetylaspartate, tetramethylamines (e.g., choline), creatine, phosphocreatine, lactate, and lipids were measured within lesions located at the brainstem using Point-resolved spectroscopy sequences. RESULTS: Diagnosis based on localized proton spectroscopy included brainstem glioma, brainstem encephalitis, demyelination, dysmyelination secondary to neurofibromatosis type 1 (NF 1), and possible infection or radiation necrosis. In all but one patient, diagnosis was confirmed by biopsy or by clinical follow-up. CONCLUSIONS: This small sample of patients suggests that MRS is important in the differential diagnosis between proliferative and nonproliferative lesions in patients without neurofibromatosis. Unfortunately, in cases of NF 1, MRS can have a rather misdiagnosis role.

Adolescent↗
PubMed2006

[Diagnostic difficulties in encephalitis and glioma].

BACKGROUND: Glial neoplasms can infiltrate the central nervous system extensively with relative preservation of the underlying neuronal architecture. The differential diagnosis between cerebral glioma and infective lesions can be very difficult to distinguish by MRI only. CASE REPORT: We report a 7 year old boy with recurrent vomiting, fever, weakness, abdominal pain and diarrhea. Besides an expressive speech disturbance the neurological examination showed no pathological findings. The sonography revealed discrete hepatomegaly and small pericardial effusion. MRI showed a diffuse mesencephalic and pontine swelling without contrast medium enhancement possibly pointing to an infective lesion. Microbiological, serological and metabolic investigations of blood and CSF were normal. After initial improvement associated with antibiotic, antiviral and dexamethasone treatment the process relapsed progessively. The 1H-MR-spectroscopy showed elevated cholin and decreased N-acetyl-aspartate levels suspicious for a proliferating process. Brain biopsy revealed anaplastic astrocytoma (WHO III). Despite of radiation and chemotherapy the tumordisease deteriorated and the patient died because of progressive brainstem infiltration one year later. CONCLUSION: This case report shows that cerebral glioma can mimick infective brain disease and that MR-spectroscopy is an important non-invasive tool in this differential diagnosis.

Aspartic Acid↗
PubMed2005

Accelerated myelination associated with venous congestion.

Magnetic resonance imaging is currently the gold standard in the assessment of brain myelination. The normal pattern of brain myelination conforms to a fixed chronological sequence. Focal accelerated myelination is a usual pathological state and previously has only been associated with Sturge-Weber syndrome. The purpose of our study is to describe alternate causes for accelerated myelination. We retrospectively reviewed serial MR scans, MR angiography, conventional angiography and the clinical progress of three children with accelerated myelination. Two patients with accelerated myelination had an underlying cerebral sinovenous thrombosis. The third patient had Sturge-Weber syndrome. Our study strongly suggests that cerebral venous thrombosis with the consequent restriction of venous outflow could be a key factor in the induction of accelerated myelination. We recommend that in patients with accelerated myelination, the search for an underlying etiology should include careful evaluation of the intracranial vascular pathology, especially cerebral venous thrombosis.

Brain↗
PubMed2005

Isolated CNS relapse in neuroblastoma.

We report three patients with primary extracerebral neuroblastoma and central nervous system (CNS) relapse. As the survival of children with metastatic neuroblastoma improves with recent advances in treatment, CNS involvement becomes more frequently detected. MR imaging of the brain and spine are strongly indicated in the follow-up of patients with increased risk of developing CNS disease.

Central Nervous System Neoplasms↗
PubMed2004

Granulocytic sarcoma in children.

We report three children with leukaemia (two acute myeloid and one acute lymphoblastic) and granulocytic sarcoma in the skull, orbit and sinuses. Lesions in these sites in children, with or without bone changes, are suggestive of systemic diseases such as lymphoproliferative conditions. Although involvement by granulocytic sarcoma, with or without acute myeloid leukaemia, is described, an association with acute lymphoblastic leukaemia is rare. Recognition of this rare entity is important, because early aggressive chemotherapy can bring about regression of the tumour and improve survival.

Child, Preschool↗
PubMed2004

Central nervous system imaging in childhood leukaemia.

The aim of this study was to document the imaging abnormalities seen in the central nervous system (CNS) in cases of childhood leukaemia or as complications of its treatment. Magnetic Resonance (MR) images and Computed Tomographic (CT) scans were reviewed retrospectively in 22 children and adolescents with neurological manifestations/complications of leukaemia or its treatment. Among the 22 patients, nine had two or more different CNS abnormalities. The imaging abnormalities seen in 15 patients before or during treatment included sinus thrombosis, cortical vein thrombosis, cerebral haemorrhage, meningeal leukaemia, infections, skull leukaemic infiltration and treatment-related neurotoxicity. After therapy, seven patients had CNS abnormalities, including secondary brain tumours, skull tumour, mineralising microangiopathy, leucoencephalopathy, transient white matter abnormalities, spinal intradural haematoma, chronic subdural haematoma, radiation necrosis, meningeal leukaemia and leukaemic infiltration at the vertebral body. CNS complications are related to the inherent risk of leukaemia itself, to the treatment method and to the duration of survival.

Adolescent↗
PubMed2002

Spinal subdural haematoma mimicking tethered cord after posterior fossa open surgery.

We report the MRI findings in a girl aged 3 years and 10 months who developed a spinal subdural haematoma after posterior fossa open surgery for cerebellar malignant rhabdoid tumour. Emergency surgery was performed immediately because of increased intracranial pressure. Control MRI 48 h after surgery showed a spinal subdural haematoma without clinical signs of paresis or bladder dysfunction. Spinal subdural haematoma is rare, and only few cases have been reported, especially in children. This report suggests that "silent" (without clinical symptoms) postoperative spinal acute subdural haemorrhage can occur after posterior fossa surgery.

Cerebellar Neoplasms↗
PubMed2002

Supratentorial leptomeningeal metastasis of a medulloblastoma without cerebellar tumor recurrence.

The authors report on a 15-year-old girl presenting with headache and nausea. Cranial magnetic resonance imaging (MRI) showed a diffuse leptomeningeal contrast enhancement 5 years after resection of a posterior fossa medulloblastoma followed by radio- and chemotherapy. A left frontal biopsy revealed tumor spread. Histopathological findings of the tumor were identical to those obtained from the original medulloblastoma. To the authors' knowledge this is the first report of late supratentorial leptomeningeal metastasis from a medulloblastoma while no local recurrence or spinal leptomeningeal dissemination had occurred.

Adolescent↗
PubMed2002

Minor head injury as cause and co-factor in the aetiology of stroke in childhood: a report of eight cases.

BACKGROUND: Traumatic stroke usually occurs after dissection of large extracranial or intracranial vessels, leading to disseminated cerebral embolism. Stretching and distorting forces in cerebral intraparenchymal end arteries can cause intimal lesions followed by an occluding thrombus. OBJECTIVE: To investigate the importance of traumatic endothelial lesions in intraparenchymal end arteries after minor head injuries. METHODS: The cases of eight children are reported. They were aged between two and seven years (mean 6.2 years), and they developed significant neurological deficits at 15 minutes to 72 hours (mean 16.3 hours) after minor head injuries. RESULTS: The the patients all had hemiparesis combined with other signs, including central facial paralysis, dysphasia, dysphagia, and extrapyramidal signs. Computed tomography or magnetic resonance imaging showed cerebral infarctions affecting branches of the middle cerebral artery (n = 3), anterior cerebral artery (n = 1), posterior cerebral artery (n = 1), and basilar artery (n = 3). These lesions affected the basal ganglia, the internal capsule, and the brain stem. Neither heart disease nor dissections of large vessels were present. Two children had prothrombotic risk factors (an increase in lipoprotein (a) and a factor V Leiden mutation). The follow up period was between three months and 13 years (mean 3.9 years). Outcome was classified according to the Glasgow outcome scale as moderate disability (n = 4), severe disability (n = 2), non-disabling sequelae (n = 1), and total recovery (n = 1). CONCLUSIONS: Minor head injuries can be cause and co-factor in the aetiology of stroke. The frequency of this may be underestimated, and detailed medical history of the days before stroke manifestation may identify more traumatic events, especially in the group of so called "idiopathic" strokes.

Accidents↗
PubMed2001

Neurological and mental outcome after severe head injury in childhood: a long-term follow-up of 318 children.

PURPOSE: Identification of prognostic risk factors in paediatric head injury. METHOD: Long-term follow-up of 318 children with severe head injuries with a mean follow-up period of 8 years and 9 months. The neurological and mental outcome was classified by the Glasgow Outcome Scale and the Frankfurt Mental Outcome Scale. RESULTS: Prognostic risk factors were a primary post-traumatic vigilance disturbance longer than 24 hours, less than seven points on the Glasgow Coma Scale, an increased intracranial pressure with cerebral perfusion pressure below 50 mmHg, age at accident younger than 2 years, physical abuse and the development of post-traumatic epilepsy. CONCLUSIONS: The infant brain is more vulnerable to lasting deficits and is more prone to post-traumatic seizure development. Post-traumatic epilepsy itself causes a deterioration in outcome because of the developmental disturbance at epilepsy onset and often unfavourable course. Physically abused children with their often multiple and repeated head injuries are predestined for poor outcome.

Adolescent↗
PubMed2001

Acute encephalopathy as a primary manifestation of haemophagocytic lymphohistiocytosis.

Haemophagocytic lymphohistiocytosis (HLH) is characterized anatomically by an infiltration of multiple tissues with lymphocytes and haemophagocytic histiocytes. First symptoms are usually hepatosplenomegaly, pancytopenia, and intractable fever. Up to 73% of those with HLH develop CNS involvement during the disease course. The peculiarity of the two patients presented here, a 20-month-old Italian female and a 4-year-old Moroccan female, is that the initial presenting neurological symptoms mimicked an encephalitis, anticipating the typical systemic symptoms by 1 and 4 months. They developed progressive encephalopathy accompanied by status epilepticus, one child developed a secondary hydrocephalus. In both children it was not possible to detect an underlying infection or malignant disease and there were no other cases in the family that suggested a familial form of HLH. Diagnosis and initiation of treatment was delayed because of the initial encephalopathic clinical picture and the late onset of the typical systemic features. As early diagnosis allows better therapeutical approaches, haemophagocytic lymphohistiocytosis should be considered in children with persistent or progressive findings of encephalopathy, especially in the absence of identification of a plausible pathogen.

Age of Onset↗
PubMed2001

Brain white-matter lesions in celiac disease: a prospective study of 75 diet-treated patients.

OBJECTIVE: Celiac disease (CD), or gluten sensitivity, is considered to be a state of heightened immunologic responsiveness to ingested gluten proteins in genetically predisposed individuals. The gastrointestinal manifestation suggests a severe enteropathy of the small intestine with malabsorption, steatorrhea, and weight loss because of a deranged mucosal immune response. Neurologic complications occur, especially epilepsy, possibly associated with occipital calcifications or folate deficiency and cerebellar ataxia. There have been reports of brain white-matter lesions as an extraintestinal manifestation in Crohn disease and ulcerative colitis but not in CD. METHODS: In this study, 75 diet-treated mainly pediatric patients with biopsy-proven CD underwent prospectively clinical neurologic examinations, laboratory investigations, electroencephalography, computed tomography, and magnetic resonance imaging. The age range was 2.8 to 24.2 years with a mean of 11.6 years. The mean period of gluten exposure was 2.4 years. RESULTS: Ten patients had neurologic findings such as febrile seizures, single generalized seizures, mild ataxia, and muscular hypotonia with retarded motor development. No folate deficiency was found. The hippocampal regions showed no abnormalities. Computed tomography did not reveal any cerebral calcifications, but magnetic resonance imaging detected unilateral and bilateral T2-hyperintensive white-matter lesions in 15 patients (20%). There was no correlation between these lesions and dietary compliance or neurologic or electroencephalographic abnormalities. The mean gluten exposure time of these patients was slightly increased (not significant). CONCLUSIONS: Focal white-matter lesions in the brain may represent an extraintestinal manifestation of CD. They may be ischemic in origin as a result of a vasculitis or caused by inflammatory demyelination. They seem to be more typical of pediatric CD than cerebral calcifications. Their prognostic value is unclear and needs to be elucidated in additional studies. CD should be suggested as a differential diagnosis in children with unclear white-matter lesions even without intestinal symptoms.

Adolescent↗
PubMed2000

Lyme borreliosis mimicking central nervous system malignancy: the diagnostic pitfall of cerebrospinal fluid cytology.

We report two children with acute loss of neurological functions and signs of an increased intracranial pressure. Imaging techniques ruled out space occupying lesions, whereas CSF cytology indicated CNS involvement of a non-Hodgkin lymphoma in the form of abnormal lymphocytic pleocytosis with malignancy criteria fulfilling lymphoid cells. CSF protein electrophoresis and Borrelia burgdorferi serology revealed neuroborreliosis which was successfully treated with antibiotic therapy. The malignancy mimicking cytology is based on a blastoid transformation of B- and T-lymphocytes due to the antigenic stimulus of B. burgdorferi infection. Lymphoid cells in the CSF of a patient with acute or chronic neurological symptoms raise the differential diagnosis of inflammatory etiology versus CNS lymphoma. Monomorphism and higher quantity of the lymphoid cells point to CNS lymphoma. A lower quantity and polyclonal pattern of lymphoid cells associated with an elevated protein fraction caused by intrathecal immunoglobulin synthesis suggest an inflammatory etiology.

Adolescent↗
PubMed2000

Midline developmental anomalies with lipomas in the corpus callosum region.

Three children with complete or partial callosal aplasia and intracranial lipoma in the corpus callosum region were investigated. Two lipomas were tubulonodular; one replaced the entire corpus callosum structure. Accompanying anomalies affected the cingulate gyrus, septum pellucidum, and choroid plexus. In one case, diagnosis was made in utero in the 25th gestational week by ultrasonography; in the second case it was made on the first day of life, also by screening ultrasonography. Two children had mild spastic distal diparesis; one complained of chronic headache. Electroencephalography showed no abnormalities; epilepsy anamnesis was negative. Somatosensory and visual evoked potentials showed prolonged conduction in two cases. Surgery was not indicated. Because of the risk of developing epileptic seizures, regular electroencephalographic follow-up investigations are essential.

Adolescent↗
PubMedDate not supplied

[Present limitations of molecular biological diagnostics in Gillespie syndrome].

BACKGROUND: Gillespie syndrome is the phenotype partial aniridia, cerebellar ataxia and mental retardation. Further malformations can be associated, mainly females are affected. Inheritance and genetics of the syndrome are unknown. Autosomal dominant aniridia is an important differential diagnosis of fixed dilated pupils and is usually associated by mutations of the PAX6 gene. In 1998 the first report of a chromosomal abnormality presenting a de novo translocation t(X;11) (p22.32;p12) detected in a patient with Gillespie syndrome has been published. PATIENTS AND METHODS: A 8 year-old girl with Gillespie syndrome phenotype associated with congenital pulmonary stenosis and helix dysplasia is reported. Karyotyping as well as molecular biological investigations of the PAX6 gene were performed. RESULTS: The karyotype of the girl and her clinically inconspicuous mother showed no abnormalities, especially no de novo translocation of the chromosomes X and 11. PAX6 gene analysis of the affected girl presented no mutations. CONCLUSIONS: The combination of muscular hypotonia and fixed dilated pupils in infancy is suspicious of Gillespie syndrome. Congenital pulmonary stenosis and helix dysplasia can be associated. PAX6 gene analysis can be helpful to distinguish between autosomal dominant aniridia and Gillespie syndrome. To illucidate the underlying genetic defects karyotyping and the search for de novo translocations especially of chromosome X and 11 should be performed.

Abnormalities, Multiple↗
PubMedDate not supplied

[Fatal outcome during physiotherapy (Vojta's method) in a 3-month old infant. Case report and comments on manual therapy in children].

Detailed clinical and neuropathological report on a fatal incident during the first manual therapy according to Vojta conducted in a 3 months old baby: during forced active rotation and head retraction the baby suffered from a bleeding into the adventitia of both her vertebral arteries at the level of C1 prompting ischemia of the caudal brainstem with subarachnoid haemorrhage around. It has to be suggested that similar cases already have occurred but have not been reported yet. There might be a time lag between the performance of physiotherapy and the beginning of neurologic symptoms. The risks of manual therapy in children will be discussed.

Brain Stem Hemorrhage, Traumatic↗
PubMedDate not supplied

[Diagnosis and outcome of neurotropic enterovirus infections in childhood].

BACKGROUND: Enterovirus infections are among the most common causes of aseptic meningitis. Worldwide there are reports about recurring outbreaks, especially during the summer. They are favoured by conditions of bad hygiene and contaminated water, transmission is predominantly through the faeco-oral route or by droplet infection. The most common species are Coxsackie B and ECHO (Enteric Cytopathogenic Human Orphan) virus. ECHO viruses have a worldwide distribution and usually occur as "summer flu" or aseptic meningitis and meningoencephalitis in toddlers and infants. Type 30 caused an outbreak of aseptic meningitis in the Rhein-Main region in summer 1997. During five months 63 children younger than 16 years were reported. PATIENTS AND METHODS: During this outbreak 18 children with prooved enterovirus infections were treated at the Frankfurt/Main University Children's Hospital. Standardized infectiological diagnostic procedures were performed and risc factors, clinical symptoms, inflammatory marker, neurophysiological findings (electroencephalography, evoked potentials) and outcome were assessed. RESULTS: The affected children were between 3 and 11 years old. Clinical symptoms were cephalgia, nausea, vomiting, meningism and seizures with fever. Virus isolation from faecal and cerebrospinal fluid (CSF) samples and the use of polymerase chain reaction (PCR) was superior to serological methods. Erythrocyte sedimentation rates showed more significant increase than C-reactive protein (CRP) and blood leukocytes. CSF pleocytosis showed high variation. Clinical course as well as prognosis and outcome were favourable. CONCLUSION: Virusisolation in stool and CSF is most promising in the diagnostic of cerebral enterovirus infections. Usually the outcome is favourable, encephalitis can occur as serious complication.

Adolescent↗