PubMed Health⌕ Search

Biomedical subjects

M J Noetzel

Publications and source records attributed to M J Noetzel.

At least 19 recordsLinked to original sources
PubMed2001

Prospective study of recovery following neonatal brachial plexus injury.

The prognosis for recovery from brachial plexus injury sustained at or before birth is generally favorable. However, roughly 10% of these infants remain profoundly weak and later exhibit functional disability in the affected arm. Early identification of these at-risk infants would be helpful in selecting patients for surgical management. In our prospective study, 80 infants with brachial plexus injury were examined on a monthly basis. Complete recovery occurred in 53 (66%); in 9 (11%), mild weakness persisted. In each child, recovery to antigravity strength in the biceps, triceps, and deltoid was noted by 6 months of age. Moderate arm weakness persisted in 7 children (9%); none had antigravity strength in the deltoid at age 6 months. Eleven children (14%) had severe permanent weakness (mean follow-up: 4.4 years). At age 6 months, these individuals exhibited at best 2/5 strength proximally and typically 0-1/5 strength in the wrist and finger extensors. Our results demonstrate that detailed strength testing up to 6 months of age predicts not only complete recovery of neonatal brachial plexus injury but also those children destined for long-term severe disability.

Birth Injuries↗
PubMed1999

Poliomyelitis-like syndrome associated with Epstein-Barr virus infection.

A 20-month-old male presented with an acute clinical syndrome resembling poliomyelitis, characterized by a flaccid monoplegia, areflexia of the involved limb, and preserved sensation. Electrophysiologic studies supported a neuronopathic localization involving the anterior horn cells. Although laboratory evidence for a poliovirus infection was absent, serologic and polymerase chain reaction studies documented an active central nervous system infection with Epstein-Barr virus, indicating that a poliomyelitis-like syndrome may be produced by infectious agents other than enteroviruses.

Diagnosis, Differential↗
PubMed1997

Serum autoantibodies in childhood opsoclonus-myoclonus syndrome: an analysis of antigenic targets in neural tissues.

OBJECTIVE: Opsoclonus-myoclonus (OM) is a rare neurologic syndrome affecting children and adults. In children it occurs as a parainfectious process or a paraneoplastic syndrome in association with neuroblastoma. Evidence for an immune mechanism includes the presence of serum autoantibodies to several neural antigens and improvement of symptoms with immunosuppressive therapy. We studied the neural antigenic targets of serum IgM and IgG autoantibodies from nine children with OM. DESIGN: We studied sera from nine children with OM, three with associated neuroblastoma and six with a prodromal viral illness. Control subjects (n = 77) included four children with neuroblastoma but not OM, 32 children with other neurologic disorders, and 41 with nonneurologic illnesses. We studied the neural antigenic targets of serum IgM and IgG autoantibodies by the following methods: (1) immunostaining of human cerebellar sections and peripheral nerve, and (2) Western blot analysis with human brain fractions including white matter, gray matter, and cerebellar Purkinje cells and nuclei. RESULTS: Sera from all nine children with OM had IgM and IgG binding to the cytoplasm of cerebellar Purkinje cells and to some axons in white matter. In peripheral nerve, IgM and IgG from all nine OM sera bound to large and small axons. Western blot analysis showed a distinctive pattern of binding to several neural proteins, including a 210 kd antigen identified as the high molecular weight subunit of neurofilament. No control serum showed a similar pattern of reactivity. CONCLUSION: Opsoclonus-myoclonus syndrome in childhood is associated with a distinctive pattern of serum IgM and IgG binding to neural tissues and antigens.

Adolescent↗
PubMed1997

Outcome of surgically treated birth-related brachial plexus injuries in twenty cases.

Birth-related brachial plexus injury occurs in 0.19-2.5 per 1,000 live births, of which 70-92% improve with conservative management. With the advent of microsurgical techniques, patients who fail expectant treatment may benefit from brachial plexus exploration and reconstruction. From 1991 to 1996, 87 patients were referred to the multidisciplinary brachial plexus clinic at St. Louis Children's Hospital. Twenty patients were selected for surgical management. The average age at surgery was 10.5 months (range 3-35, median = 8), with an average follow-up of 23.9 months (range 7-45, median = 24). Two patients were lost to follow-up. Surgical procedures included neurolysis (n = 8), neurotization (n = 2), nerve grafting (n = 5), and a combination (n = 3) of the above. Two patients underwent exploration without repair. Intercostal nerves, pectoral nerves, and C4 roots were used for neurotizations, and the sural nerve was used for nerve grafting. Results from 18 patients were available for follow-up review. Fifteen patients (83% demonstrated clinical improvement postoperatively. Of the 3 patients without improvement, 2 underwent exploration without repair, and one underwent neurolysis of the axillary nerve. Of patients undergoing reconstruction, 93% had improved strength postoperatively. No subjects had worsening neurologic status, and there were no complications. These results suggest that surgery for birth-related brachial plexus injury may show favorable outcomes if patients are selected appropriately. Patients undergoing neurolysis and nerve grafting had more favorable outcomes than those undergoing neurotization.

Brachial Plexus↗
PubMed1996

Long-term studies of metopic synostosis: frequency of cognitive impairment and behavioral disturbances.

Although the occurrence of cognitive impairment and behavioral disturbances in patients with metopic synostosis has been described, the incidence of this dysfunction has not been established. The records of 36 consecutive children with metopic synostosis followed at one craniofacial center from 1978 to 1993 were reviewed and parental questionnaires were completed to establish the frequency of mental retardation, learning disabilities, and behavioral problems associated with this synostosis. Documentation of syndromes, abnormal karyotype, and central nervous system anomalies also was done. The study group consisted of 27 males and 9 females. The average age at most recent follow-up was 7 years and 1 month (range 6 months to 22 years). Two patients had chromosomal abnormalities (9p syndrome and trisomy 21). On the basis of CT and MRI scans, intracranial anomalies were identified for only one patient having an absent corpus callosum. Thirty-two of the study patients had adequate information for longitudinal assessment. Twenty patients have normal development without apparent disability. Of these, those of school age are at appropriate grade level. Eight patients have mild to moderate learning disabilities or behavioral problems, including attention deficit/hyperactivity disorder and impaired language development. Four patients have significant mental impairment. Impaired cognitive development was not limited to children with abnormal karyotype or central nervous system anomaly. Cognitive and behavioral abnormalities occur in at least a third of patients with metopic synostosis. The, at times, subtle nature of these abnormalities mandates longitudinal developmental and neurologic evaluation for infants with metopic synostosis.

Adolescent↗
PubMed1995

Cerebrospinal fluid 5-hydroxyindoleacetic acid and homovanillic acid in the pediatric opsoclonus-myoclonus syndrome.

To study the purported role of central monoamine disturbances in the pathophysiology of the opsoclonus-myoclonus syndrome, the serotonin metabolite 5-hydroxyindoleacetic acid and the dopamine metabolite homovanillic acid were measured in cerebrospinal fluid samples from 27 affected children and 47 age- and gender-matched control subjects by high-pressure liquid chromatography with electrochemical detection. 5-Hydroxyindoleacetic acid and homovanillic acid concentrations in the cerebrospinal fluid were approximately 30 to 40% lower in opsoclonus-myoclonus patients compared to control subjects, and the normal inverse correlation between age and monoamine metabolite concentrations in the cerebrospinal fluid of control subjects was not found in opsoclonus-myoclonus patients. Patients with the lowest values were less than 4 years old, and a subgroup had extremely low levels, but differences in older children were not significant. Cerebrospinal fluid levels of 5-hydroxyindoleacetic acid and homovanillic acid were more positively correlated in control subjects than in opsoclonus-myoclonus patients. None of the patients exhibited high levels of monoamine metabolites. Homovanillic acid levels were slightly lower in the cerebrospinal fluid of patients receiving corticotropin or steroids at the time of lumbar puncture. Clinical variables that could be excluded were paraneoplastic etiology, anesthetic for lumbar puncture, syndrome duration, age at onset, gender, response to steroids, length of time until initiation of corticotropin or steroids, presence of seizures, opsoclonus, and functional impairment. These data suggest a disturbance and possible altered ontogeny of serotonin or dopamine neurotransmission in a subpopulation of children with opsoclonus-myoclonus with low cerebrospinal fluid levels of 5-hydroxyindoleacetic acid and homovanillic acid.(ABSTRACT TRUNCATED AT 250 WORDS)

Adrenal Cortex Hormones↗
PubMed1995

Triopia: craniofacial malformation with prosencephalic duplication.

A case of ocular duplication with complex craniofacial and central nervous system anomalies is described. The anomaly is termed triopia because the child's most overt and distinguishing feature was three eyes: the left orbit contained two globes with independent ocular adnexa; the right orbit contained one normal appearing and functioning globe. Computer assisted medical imaging was used to define, in vivo, the intra- and extracranial soft and hard tissue anomalies: the cerebral hemisphere ipsilateral to the ocular duplication was also duplicated. Possible bases for this anomaly include duplication of primordia for the eye and secondary prosencephalon.

Abnormalities, Multiple↗
PubMed1995

Changes in hip migration after selective dorsal rhizotomy for spastic quadriplegia in cerebral palsy.

Selective dorsal rhizotomy is increasingly used for management of spastic quadriplegic cerebral palsy but rates of hip stability following the operation have not been reported. Determining hip stability by radiographic measurement of lateral migration of the femoral head beyond a lateral edge of the acetabulum after dorsal rhizotomy allows an objective assessment of the outcome of the operation. This prospective study examined the effect of selective dorsal rhizotomy on lateral migration of the femoral head in 45 children with spastic quadriplegic cerebral palsy. The children ranged in age from 2 to 9 years (average 5 years 1 month) and were grouped according to their ages with 23 children in the 2- to 4-year-old group and 22 children in the 5- to 9-year-old group. Postoperative follow up ranged from 7 to 50 months (average 20 months). The Reimers migration percentage (MP), a measure of the lateral migration of the femoral head, was calculated from anteroposterior hip radiographs taken prior to the operation and at the last follow-up examination. Of the 90 hips involved, 9% improved, 80% remained unchanged, and 11% worsened, yielding a radiographic stability rate of 89%. The hips with postrhizotomy worsening of the MP had an average preoperative MP of 14% (range 9% to 38%) and an average postoperative increase in MP of 18% (range 11% to 37%). Of the 45 children, four subsequently underwent unilateral derotational femoral osteotomies for persistent or worsening hip subluxation. There was a significant tendency for the MP to worsen in patients with lower prerhizotomy MP values (chi 2 = 20.74, df = 4, p = 0.001), but the age of patients and their ambulatory status at the time of rhizotomy had no bearing on postoperative hip stability. The data indicate that selective dorsal rhizotomy prevents progressive lateral migration of the femoral head in the majority of children who undergo the operation for spastic quadriplegia.

Cerebral Palsy↗
PubMed1995

Fast spin-echo magnetic resonance imaging for radiological assessment of neonatal brachial plexus injury.

Neurosurgical management of birth-related brachial plexus palsy involves observing the patient for a period of several months. Operative intervention is usually undertaken at 3 to 6 months of age or more in infants who have shown little or no improvement in affected muscle groups. Ancillary tests such as electromyography and nerve conduction studies are occasionally useful. No radiological study has been consistently helpful in operative planning, except for contrast computerized tomography (CT) myelography, which requires general anesthesia in infants. This is because the infant's small size exceeds the functional resolution of the imaging modalities. This report describes the use of a special sequence of magnetic resonance (MR) imaging entitled "fast spin echo" (FSE-MR). Unlike CT myelography, this technique provides high-speed noninvasive imaging that allows clinicians to evaluate preganglionic nerve root injuries without the use of general anesthesia and lumbar puncture. The utility of this technique is illustrated in three cases, two involving either infraclavicular exploration or a combination of infraclavicular and supraclavicular exposure based on FSE-MR findings. The FSE-MR imaging offers an excellent alternative to contrast CT myelography in evaluation of infants with birth-related brachial plexus injuries.

Birth Injuries↗
PubMed1992

Seizures in children with congenital hydrocephalus: long-term outcome.

We documented seizures in 33 of 68 (48.5%) children with congenital hydrocephalus not associated with myelomeningocele. Mental retardation (MR) and CNS malformations correlated with seizure occurrence; age at shunt insertion and number of shunt revisions and infections were not significant variables in predicting seizures. Of 11 patients seizure free for 2 or more years on medication, six had therapy discontinued without seizure recurrence. Among those 33 children with seizures, 14 (42.4%), including five who had failed withdrawal of medication, have adequately controlled seizures on anticonvulsants. Frequent convulsions despite treatment occur in 13 (39.4%) of the 33 children with seizures. Absence of MR, older age and nonparoxysmal EEG at seizure onset, and absence of CNS malformation correlated with seizure remission. Longer time without seizures while on medication did not predict successful discontinuation of therapy. In contrast, MR correlated significantly with seizure recurrence following cessation of treatment. Our study indicates that medication can be safely discontinued in children with congenital hydrocephalus who are of normal intelligence and have been seizure free on anticonvulsants for 3 years.

Analysis of Variance↗
PubMed1991

Prognosis for seizure control and remission in children with myelomeningocele.

A combined retrospective and prospective study was designed to determine the incidence of seizures in 140 children with myelomeningocele, as well as the potential for seizure control and remission. The incidence of seizures in 109 patients with myelomeningocele and hydrocephalus was 16.5 per cent, and 19.4 per cent in a further 31 patients without hydrocephalus. Mental retardation, often in combination with cerebral malformations, was significantly more common in children with seizures, regardless of presence or absence of hydrocephalus. Of the 24 patients with convulsion, three-quarters had anti-epileptic medication discontinued, without recurrence of seizures. An additional five children's seizures are well controlled with medication. Mental retardation was the only significant predictor of long-term outcome. These results indicate that children with myelomeningocele have an excellent prognosis for seizure control and subsequent remission off medication.

Anticonvulsants↗
PubMed1990

Synthesis and phosphorylation of the glial fibrillary acidic protein during brain development: a tissue slice study.

Brain slices were incubated with either [3H] amino acids or [32P] orthophosphate in order to characterize the synthesis and phosphorylation of the glial fibrillary acidic protein (GFAP) in the rat nervous system. The incorporation of [3H] amino acids into GFAP was found to increase significantly during early postnatal development, reaching a peak of activity on day 5 of life and then declining over the next 2 weeks. Concomitant with this peak of synthetic activity the content of GFAP in rat brain was also observed to increase dramatically. GFAP continued to accumulate in brain through postnatal day 30 despite a decrease in the synthesis of the protein. These results indicate that the increase in GFAP during the first month of life cannot be ascribed solely to the rate of GFAP synthesis. The findings are consistent with the hypothesis that during later stages of astrocytic development the accumulation of GFAP may be primarily dependent upon a low rate of protein degradation. The pattern of GFAP phosphorylation in the developing rat brain differed from that observed for the incorporation of [3H] amino acids. The peak incorporation of 32P into GFAP occurred on postnatal day 10 at a time when synthesis of the protein had declined by 43%. These findings suggest that during development phosphorylation of GFAP is mediated by factors different from those directing its synthesis. In addition, phosphorylation of GFAP did not alter its solubility in cytoskeletal preparations indicating that GFAP phosphorylation is probably not a major regulatory mechanism in disassembly of the astroglial filaments.

Amino Acids↗
PubMed1990

Phosphorylation of the glial fibrillary acidic protein.

The glial fibrillary acidic protein (GFAP) was found to be phosphorylated in vivo after intracerebral injection of [32P]-orthophosphate, in brain slices, and in a cell free system. The phosphorylated proteins were separated by two-dimensional gel electrophoresis and then transferred to nitrocellulose sheets. Two isoelectric variants of GFAP were immunochemically identified by monoclonal antibodies. Autoradiography demonstrated that only the more acidic isoelectric variant of GFAP was phosphorylated. Phosphoamino acid analysis revealed that under all conditions GFAP was phosphorylated at serine and threonine residues. Incubation of brain slices with [32P]-orthophosphate and the protein kinase C activator phorbol 12-myristate 13-acetate or forskolin, an activator of cyclic AMP-dependent protein kinase, stimulated phosphorylation of GFAP. Likewise phosphorylation of GFAP was also accentuated by calcium/phosphatidylserine/diolein and by exogenous cyclic AMP-dependent kinase in a cell free system. These findings announce that protein kinase C and cyclic-AMP dependent kinase may play physiologic roles in the in situ phosphorylation of GFAP. When isolated cytoskeletal preparations were incubated with [gamma-32P] ATP, GFAP was phosphorylated in vitro by two additional protein kinases, a Ca2++/calmodulin-dependent kinase and an effector-independent kinase. The results of these investigations strongly suggest that phosphorylation of GFAP appears to be regulated by multiple second messenger pathways.

Animals↗