Threatening spinal cord compression during anesthesia in a child with mucopolysaccharidosis VI.
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Anesthesia, Dental↗
Biomedical subjects
U Stephani
Publications and source records attributed to U Stephani.
Electrolytes, amino acids and proteins in lumbar CSF during the treatment of acute leukemia in childhood.
We performed analyses of electrolytes, amino acids, albumin, alpha 2-macroglobulin, gamma-globulin and LDH in the lumbar cerebrospinal fluid of children undergoing treatment for acute lymphoblastic leukemia, non-Hodgkin-lymphoma or acute myeloid leukemia. At the time of diagnosis signs of a disturbance of the blood-brain barrier were found in some patients. During induction treatment with L-asparaginase a rise of glutamic acid and a decrease of glutamine occurred. This finding correlated with slowing of the EEG. Treatment with vincristine was associated with a slight drop of sodium and chloride concentration in serum, but not in the cerebrospinal fluid. Central nervous system prophylaxis with cranial irradiation, and to a lesser degree with intravenous medium-dose methotrexate, gave rise to a further deterioration of the blood-brain barrier function as indicated by an increase in albumin, alpha 2-macroglobulin and LDH levels. During radiotherapy the concentration of several amino acids rose, probably due to a disturbance of active carrier mechanisms. Patients with elevated albumin at the end of radiotherapy more often suffered an early leukemia relapse while still on treatment. No other clinical or electroencephalographic correlations of altered barrier function could be found.
Amino Acids↗
[Conservative treatment of non-resorptive hydrocephalus in premature infants].
Following perinatal asphyxia and intracranial hemorrhage frequently progressive ventricular dilatation develops in preterm infants. Most common is communicating hydrocephalus due to obliterative arachnoiditis. Ventricular dilatation is reported to affect normal brain development and early therapy is recommended. Cerebrospinal fluid shunting is still accompanied by multiple complications, esp. in preterm infants with a birth-weight below 1,500 g. Seven preterm infants, born between the 27th and 34th gestational week with a birthweight of 910-1,940 g were medically treated for their progressive communicating hydrocephalus. The therapy consisted of intermittant lumbar punctures, medication of acetazolamide and furosemide as well as electrolyte and base replacement. Therapy was started at the 14th-31st postnatal day and lasted from 46 to 149 days. In all children the ventricular dilatation diminished. A steady state of cerebrospinal fluid production and absorption was regained in four children. Due to reoccurrence of ventricular dilatation shunting was performed in three others at the age of more than 3 months and with a weight of 3,620-5,170 g. Thus, medical therapy of hydrocephalus provides time for development of preterm infants, delay of shunting procedures and normalisation of cerebrospinal fluid dynamics.
Acetazolamide↗
Nerve growth factor (NGF) in serum: evaluation of serum NGF levels with a sensitive bioassay employing embryonic sensory neurons.
Considerable controversy surrounds the question of whether or not nerve growth factor (NGF) or a related nerve growth-promoting factor is present in serum. Recently, supporting its role as a local neuronotrophic factor, the presence of NGF in glial cells and its production in target tissues of NGF-responsive neurons were demonstrated [Rush: Nature 312:364-367, 1984; Heumann, Korsching, Scott, Thoenen: EMBOJ 3:3183-3189, 1984; Shelton and Reichardt: Proc Natl Acad Sci USA 81:7952-7955, 1984]. At the same time, the concept that NGF may play a role as a humoral factor has been questioned, since careful analyses of serum with specific and sensitive radioimmunoassays [Suda, Barde, Thoenen: Proc Natl Acad Sci USA 75:4042-4046, 1978; Korsching and Thoenen; Proc Natl Acad Sci USA 80:3513-3516, 1983; Furukawa, Kamo, Furukawa, Akazawa, Satoyoshi, Itoh, Hayashi: J Neurochem 40:734-744, 1983] as well as bioassays [Skaper and Varon: Exp Neurol 76:655-665, 1982] have not confirmed earlier reports [Levi-Montalcini and Booker; Proc Natl Acad Sci USA 46:373-391, 1960; Banks, Banthorpe, Charlwood, Pearce, Vernon: Nature 246:503-504, 1973; Hendry: Biochem J 128:1265-1272, 1972] on NGF's representation in serum. In this study serum from mouse, rat, and man was analyzed with an in vitro bioassay system which employs sensory neurons from chicken embyro dorsal root ganglia and which allows the measurement of NGF concentrations as low as 0.8 pM. It was found that sera from all these species contained neuronotrophic activity (S-NGF). The target cell spectrum as well as characteristic parameters of the neuronal growth response of S-NGF and of NGF were identical. S-NGF of mouse serum was completely inhibitable by polyclonal and monoclonal antibodies to mouse submandibular gland beta NGF. On polyacrylamide isoelectric focussing gels mouse and human S-NGF could be recovered from the same position as NGF as well as from the region where alpha 2-macroglobulin and serum albumin focused. In newborn and adult male and female mice basal S-NGF levels were equivalent to 10-50 pM NGF. A fraction of the serum samples of male mice showed elevated S-NGF levels. The incidence of high S-NGF levels was more frequent in NMRI and C57BL/6 males than in BALB/c males. Following sialectomy of male mice only basal S-NGF levels were observed up to 5 weeks after the operation. This indicates that although the submandibular gland may contribute to S-NGF levels in serum under certain conditions that appeared to be stress related, it cannot be the only source of S-NGF.
Animals↗
Slight impairment of psychomotor skills in children after treatment of acute lymphoblastic leukemia.
Several studies have reported a decline in intelligence and cognitive functions in survivors of childhood acute lymphoblastic leukemia (ALL). Other investigators, however, have found no intellectual impairment in these children. Fifty-one long-term survivors of ALL, having been treated according to the protocols of the BFM Study Group from 1970 to 1979, were assessed retrospectively using neurophysical methods. The results were compared with those obtained from 30 patients with other malignancies, who had received neither radiation therapy to the central nervous system (CRT) nor any methotrexate during chemotherapy. Additionally, neurological examinations and cranial computed tomography (CCT) were performed. neuropsychological examinations included verbal functions, intelligence (performance), psychomotor speed, motor skills and sensory integration. The results of verbal tests and the IQs, tested by nonspeed-related measures, were within normal limits in both groups. About one-third of all patients showed mild disturbances of psychomotor speed and motor skills. Children with leukemia had lower scores than those with solid tumors for nearly all tasks, but only tests for sensory integration revealed significant differences between former ALL patients and tumor patients. Furthermore, the following results were obtained related to different therapeutic modalities: The higher total radiation doses had been during CRT (maximum 24 GY), the more neuropsychological functions were impaired, particularly motor accuracy and sensory integration. These disturbances improved with the length of survival. Widening of subarachnoidal space was found in 33% of the CCT obtained. There was no correlation between the intellectual functions of the survivors and the CCT abnormalities. Neurological findings mainly consisted of slight fine motor disturbances.(ABSTRACT TRUNCATED AT 250 WORDS)
Achievement↗
Radiation-related disturbance of blood/brain barrier during therapy of acute lymphoblastic leukaemia.
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Blood-Brain Barrier↗
Cranial computed tomography of 64 children in continuous complete remission of leukemia I: relations to therapy in modalities.
64 children with acute lymphoblastic leukemia and non Hodgkin's lymphoma in continuous complete remission were studied by cranial computed tomography two months to 7 years after cessation of antileukemic treatment. 17 (27%) patients showed abnormal widening (grade 2) of external cerebrospinal fluid spaces, 20 (31%) minimal widening (grade 1). A significant correlation between grade 2 abnormalities and the duration of maintenance therapy and single cranial radiation dose was found, whereas the correlations with total radiation dose and with intensity of induction protocols were not significant. Widening of external cerebrospinal fluid spaces after cessation of therapy persisted.
Acute Disease↗
Cranial computed tomography of 64 children in continuous complete remission of leukemia II: relations to patient data and neurological complications.
Abnormal widening of cortical sulci as seen in posttherapeutic cranial computed tomography (CCT) of 64 children in complete continuous remission (CCR) of acute lymphoblastic leucemia (ALL)/non Hodgkin's lymphoma (NHL) was related to patient data and neurological complications during the application of West Berlin treatment protocol. Age and neurological/neurodevelopmental findings of the patients at diagnosis of their disease positively and significantly correlated with CCT abnormalities. Abnormal pre- and intratherapeutic electroencephalographic (EEG) recordings, the development of a severe polyneuropathy syndrome and/or a considerable loss of weight during treatment were frequently associated with an abnormal widening of cortical sulci. Occurrence of transient early encephalopathy syndrome, development of radiation induced blood brain barrier disturbance and somnolence syndrome were not correlated with these CCT changes. Possible explanations of these abnormalities are discussed.
Acute Disease↗
Successful treatment of pulmonary and cerebral aspergillosis in an immunosuppressed child.
A favourable outcome was observed in a 12 year-old boy who developed invasive pulmonary and cerebral aspergillosis during antineoplastic treatment for central nervous system relapse of acute lymphoblastic leukemia. Combination therapy with amphotericin B and 5-Fluorocytosine led to complete regression of pulmonary infiltrates. Despite enlargement of the cerebral lesion monitored by computerized tomography, no viable fungi were found in the completely resected abscess after a 4 weeks' course of antifungal treatment preceding neurosurgery. Histological examination confirmed the diagnosis of an aspergillotic abscess. The initially severe neurological symptoms disappeared after successful surgery. Aspergillus fumigatus was detected in the soil of a potted ornamental plant in the mother's living room, suggesting that this might have been the source of the infectious agent.
Amphotericin B↗
Rare complications in a case of generalized meningococcal disease: immunologic reaction versus bacterial metastasis.
A 14-year-old girl was suffering from meningococcal sepsis with initial endophthalmitis. During a secondary and prolonged fever attack including aseptic meningitis, temporary complications such as pericarditis, arthralgic pains, headache and localized phlebitis were observed. There were three relapses of pericarditis within nine months. Their course was benign. The discussion deals with the pathogenesis of these complications as metastatic and immunologic reactions. The possibility of these complications should be considered during diagnostic investigation and therapeutic treatment.
Adolescent↗
[Brain stem encephalitis (author's transl)].
Basing on a case of brain stem encephalitis in a girl of 7 1/2 years of age, the features of this disease pattern are described. The initially found disturbance of the blood brain barrier provides a clue to the possible pathogenesis of the disease in association with the pattern of clinical signs, namely, an inflammatory-hyperergic reaction of the neurovascular system in the brain stem.
Blood-Brain Barrier↗
[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↗
[Opitz-trigonocephaly syndrome--a characteristic dysmorphia-retardation syndrome of unclear origin].
Report on a patient with Opitz-Trigonocephaly-Syndrome who was diagnosed at the age of ten months. He had a trigonocephaly and multiple other stigmata including oblique orbital fissures, internal strabism, high and narrow palate with broad alveolar ridges, microstomia, large dysplastic ears, nuchal cutis laxa, broad clumsy fingers. Additionally he had a ventricular septal defect, was dystrophic and developmentally retarded. Chromosomal analysis was normal. A sister that was recently born showed no dysmorphic signs. So far, 24 cases have been reported in the literature. This is the 5th patient in Germany.
Abnormalities, Multiple↗