Search PubMed⌕ Search

Biomedical subjects

G Kurlemann

Publications and source records attributed to G Kurlemann.

90 records · Page 5Linked to original sources

[Optimizing epilepsy therapy in children and adolescents with lamotrigine].

Lamotrigine is a broadly effective antiepileptic drug in mono- and add-on therapy for children and adolescents with focal and generalized epilepsies. Some epileptologists consider lamotrigine as the drug of primary choice in older school children and adolescents because of its good tolerability (no increase of body weight, no impairment of cognitive functions, due to new data probably no teratogenic properties). Lamotrigine can be used with good efficacy in numerable epilepsy diseases, such as tuberous sclerosis, juvenile neuronal lipofuscinosis and Rett syndrome. The first studies show that lamotrigine is also effective in children under 2 years of age. For therapy of difficult-to-treat epilepsies the combination of lamotrigine with valproate has proved as especially useful. This clinical observation is supported by new results of animal experiments. The dose-dependant and typical CNS side effects vertigo, ataxia, nausea, tremor and diplopia are found most frequently. The rate of allergic skin rashes which was very high before 1998 has decreased markedly by new dosage guidelines and is now as low as in older antiepileptic drugs. Lamotrigine does not impair cognitive functions, especially not memory and language. It has mood-stabilizing features and may improve quality of life. In animal experiments lamotrigine shows antiepileptogenic and neuroprotective effects.

Adolescent↗

[Benign intracranial hypertension in childhood--pseudotumor cerebri].

Benign intracranial hypertension-pseudotumor cerebri (PTC) is a rare disease in childhood. We report about our experience in five children with PTC, aged two to ten years. The main symptoms were headache, palsy of VI.th cranial nerve and papilledema. Neuroradiologic studies showed normal ventricles in four and a slit ventricle in one. Enlarged optic nerve sheaths were found in one child prior to therapy. The cerebro spinal (CSF) fluid pressure varied from 380 mm to 480 mm CSF. Four children received acetazolamide, one child dexamethasone. Permanent visual deficit did not occur in any child.

Acetazolamide↗

[Serum prolactin after cerebral and psychogenic seizures in childhood and adolescence--an additional useful method for differentiating the two forms of seizure].

Prolactin levels were measured immediately after the seizure in some, and 15 to 20 minutes later in all of 67 children aged between 6 months and 17 years. Values were determined after grand mal, complex partial and petit mal seizures and psychogen seizures. A more than 2 to 3 fold prolactin increase over the baseline value occurred almost always after grand mal and regularly after complex partial seizures. No hyperprolactinaemia was observed after petit mal seizures. Also after psychogenic seizures a rise in serum prolactin failed. The neurophysiological basis underlying this phenomenon is a decrease of gaba- und dopaminergic systems associated with the seizure. The described method is useful in the differential diagnosis of epileptogenic versus psychogenic seizures.

Adolescent↗

[Prolactin--a diagnostic aid in cerebral seizures].

Prolactin concentration was measured 20 minutes after each seizure in 8 patients with grand mal, 2 patients with complex partial seizures and 5 patients with petit mal seizures. In the group of grand mal and complex partial seizures serum prolactin showed markedly increased levels. After petit mal seizures there was no change in serum prolactin concentration. The possible causes for changes in serum prolactin after a seizure in children are discussed.

Adolescent↗

[Traumatic macroglossia--a rare indication for tracheotomy].

Hemorrhage into the tongue caused by a trauma is able to produce dangerous hematomas. During an hypertensive crisis a 17 year old patient suffered from an ictus with a bite on his tongue. A macroglossia developed with shortness of breath, so that only a tracheotomy could grand a sufficient ventilation. The macroglossia decreased within 6 days, the nasal breathing was no longer prevented, so the tracheostoma could be closed. An operative intervention is necessary, if conservative therapy is without success referred to diminuation of the tongue.

Adolescent↗

[Plexiform neurofibroma and basal ganglia anomaly in Watson syndrome].

A 4 year-old boy was referred for diagnostic reevaluation with known pulmonary valve stenosis. Physical examination revealed multiple cafe-au-lait spots, inguinal freckling and on the right side in supraclavicular region a softly, non-painful tumour. The boy showed a mild mental and language retardation. Ultrasound and MRT demonstrated supraclavicular a plexiform neurofibroma and intracranial increased intensity lesions in basal ganglia and mesencephalon. In our patient, we have diagnosed a Watson-Syndrome, the overlap and differences to neurofibromatosis type I is discussed.

Basal Ganglia↗

[Tuberculous meningitis in a 13-month-old boy: a case report].

We present the case of a 13-month old Turkish boy of Kurdish origin with tuberculous meningitis. Fever of unknown origin and neurologic symptoms (loss of ability of walking and free sitting, cerebral seizures, central paresis of the VII. cranial nerve, coma) led to the diagnosis. Cranial CT demonstrated hydrocephalus and enhancement of the basal meninges after contrast injection; the chest x-ray showed an infiltrate in the right upper lobe of the lung and the cerebrospinal fluid (CSF) mild pleocytosis with elevated protein and reduced glucose concentrations. Diagnosis was confirmed by detection of Mycobacterium tuberculosis in the CSF by polymerase chain reaction (PCR). Immediately, surgical and level-controlled tuberculostatic treatment was initiated. The patient recovered completely.

Antitubercular Agents↗

Monitoring tumor activity in low grade glioma of childhood.

Chemotherapeutic or radiotherapeutic regimens are being increasingly used in low grade glioma of childhood. These protocols require methods to monitor tumor activity. We report our experience in eleven patients. The tumors were localized in the optic pathway (3), cerebral cortex (4) and thalamus/hypothalamus (4). Histological diagnoses included low grade astrocytoma (6), gliofibroma (1) and ganglioglioma (2). Two children with neurofibromatosis type 1 (NF-1) and typical optical tumors were not biopsied. 13 episodes of progression were noted including 3 altered diagnoses. This was evident from clinical symptoms in 11/13 episodes, computed tomography (CT) or magnetic resonance imaging (MRI) in 10/13 situations, iodine-123-alpha-methyltyrosine (123I-IMT) single-photon emission computed tomography (SPECT) in 10/10 situations, fluorine-18 fluorodesoxyglucose (18F-FDG) positron emission tomography (PET) in 0/3 and thallium-201 (201Tl) SPECT in 1/1. Seven responses to chemotherapy were recorded. Clinical symptoms indicated this in 7/7 situations, MRI in 5/7, 123I-IMT SPECT in 1/2 and 201Tl SPECT in 1/1. These data suggest that 123I-IMT SPECT is a valuable addition to low grade glioma diagnostic and stress the need for a prospective study.

Adolescent↗

[Sinus histiocytosis with massive lymphadenopathy with complete occlusion of the superior and inferior vena cava].

Sinus histiocytosis with massive lymphadenopathy (SHML) is a rare disease of the lymph nodes, still of unknown origin. We are reporting the case of a 16 year old boy with SHML which occurred in 1983. Investigations showed a massive lymphadenopathy of the mediastinal and abdominal nodes, causing displacement and compression of surrounding tissue. The patient further developed a blockage of the vena cava superior and inferior, leading to numerous collateral circulation routes in the upper and lower extremities. The etiology of the venous blockage is still disputed. It is possible that they are the result of compression of the major veins. Alternatively, the cause could lie in the disruption of the coagulation system. Finally and more likely, the problem could be the result of fibrosis developing through the healing process.

Adolescent↗

[Arteriovenous malformations of the cervical spinal cord].

Arterio-venous malformations (AVM) of the CNS are considered uncommon lesions in childhood, but is a main cause for subarachnoid haemorrhage (SAH) of children. We report a case of a 14-year-old girl with an AVM localized in the cervical spine: after an acute event with SAH she shows the clinical features of a cervical myelopathy-syndrome. MRI and selective angiography show an AVM, originating from the right vertebral artery. Patients with AVM have a higher incidence of recurrent bleeding with SAH, hematomyelia or infarction and even cord compression. This grave sequel makes it necessary to treat AVM e.g. with embolization.

Adolescent↗

[Kohlschütter syndrome--an example of a rare progressive neuroectodermal disease. Case report and review of the literature].

Kohlschütter's syndrome is a combination of amelogenesis imperfecta, progressive mental retardation and epileptic seizures. We report on a patient with typical signs of this syndrome. Beneath severe enamal defects of teeth, the patient has been suffering from progressive mental and motoric retardation from the age of six months. Although there is pathologic activity in EEG, seizures have not yet appeared. MRT shows distinct signs of cerebral atrophy. Apart from this patient 15 patients in 4 families have been reported up to now in literature. The article compares diagnostic results in this case with those reported in literature.

Amelogenesis Imperfecta↗

[Autosomal dominant cerebral seizures in the 6th month of life with benign outcome].

We report a boy with benign familial seizures of the sixth month of life. At the age of six months he suffered his first afebrile grand mal seizure. Up to his ninth month several seizures of the same type occurred. EEGs were always normal. The history yielded a paternal hereditary trait: five members of the family had also suffered grand mal seizures in the sixth month of life. All but one had no therapy with antiepileptic drugs. One girl was treated with phenobarbital for two years. All members of the family show normal neurological development. Under low dose therapy with phenobarbital our patient has remained seizure-free up to now.

Chromosome Aberrations↗

[The neurotic conversion disorder spectrum in neuropediatrics].

The incidence and symptomatology of conversion disorders in a neuropaediatric department was described. One year was retrospectively evaluated. From totally 12 patients with conversion disorders 7 were suffering for an impaired walk and each 1 patient for visual defect, choreoathetosis and confusion, convulsive seizures, tic, paralysis. "Conversion disorder" is an important and often ignored diagnosis in neuropaediatrician.

Adolescent↗

[Heterotopic gray matter: MR findings and clinical aspects].

Heterotopias are conglomerates of neurons and glial cells in an abnormal location and belong to a group of malformations, which are the result of a disturbed migration of neurons during the embryonic development of the brain. The MRI-appearance and clinical symptoms of 14 patients with nodular or lamellar heterotopias are presented. Seizures were the leading symptom (12 of 14 patients), in children also a developmental deficit (4/10) with or without seizures. Most of the children (9/10) had further brain malformations especially of the corpus callosum or the cerebellar vermis. Nodular heterotopias without further malformations were found in 4 patients, two of them developing grand-mal seizures after childhood. On MRI heterotopias are characterized by a signal isointense to gray matter in all sequences. MRI-scans in at least 2 orientations are necessary to detect these anomalies reliably. MRI is the optimum method for the demonstration of these anomalies and should always be performed if seizures develop during childhood. At least in nodular heterotopias there is no definite correlation between the extent of the anomalies, the EEG-findings and the clinical symptoms. However there is a frequent combination of heterotopias with further cerebral malformations.

Adolescent↗

[Neuralgic shoulder amyotrophy as differential diagnosis of scapula alata].

We describe two patients with neuraligic amyotrophia of the shoulder: a 15 year old boy and a girl which was 6 years old at the time of primary manifestation. Mostly adolescent patients are concerned. Beginning is acute, the etiology unclear. The patients initially feel intensive pain in one shoulder, which typically is followed by motorical weekness of the proximal upper arm and the shoulder. Nearly all patients reach a restitutio ad integrum after some months. As differential diagnosis we have to take into account the facio-scapulo-humeral muscular dystrophy, pareses of single or various nerves or a neurititis of infectious origin.

Adolescent↗

[EEG in diagnosis of other disease pictures than epilepsy].

Electroencephalography plays a very important role in the diagnosis and course monitoring of epilepsy. The EEG is also able to give decisive clues in diseases other than epilepsy. It is important to know these specific EEG patterns which may assume a key role in the diagnosis of rare neuropediatric diseases. Familiarity with such specific patterns will allow to select only specific investigations for diagnostic confirmation. We report six guiding EEG patterns and the associated disorders: 1. Re-build up phenomena for Moyamoya syndrome. 2. High amplitude alpha-beta activity for lissencephaly type I. 3. Positive spikes during low frequency photostimulation for late infantile neuronal ceroid lipofuscinosis. 4. Periodic high amplitude discharges for subacute sclerosing panencephalitis (SSPE). 5. Burst suppression pattern in non-asphyctic mature newborns for Ohtahara syndrome, non ketotic hyperglycinemia or molybden cofactor deficiency. 6. High amplitude 3-4 HZ activity with small spikes for Angelman syndrome.

Adolescent↗

Melatonin in epilepsy: first results of replacement therapy and first clinical results.

At a single evening dose of 5-10 mg, melatonin (MLT), the pineal gland hormone, can exert a positive effect on the frequency of epileptic attacks in children with sleep disturbances of various etiologies. We have shown that the sleep behavior can be normalized and an existing epilepsy can be favorably influenced. Pretherapeutic MLT secretion profiles can provide new information concerning the origin and treatment of these disturbances. In vitro experiments suggest that this effect might be the result of the interaction between MLT and MLT-specific receptors in the neocortex. Due to its favorable safety profile, MLT can be liberally administered in the specified doses and be considered as a useful antiepileptic drug.

Anticonvulsants↗