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Predictive factors of short-term neurologic outcome in children with encephalitis.

When a child is admitted to the hospital with presumed encephalitis, the physician must use clinical criteria to gauge the appropriate level of hospital care and to give a preliminary assessment of outcome to the family because the etiology is unknown. This study attempted to determine which clinical factors gathered on hospital admission would be most helpful to the physician. The records of 106 children (ages 1 month to 20 years), admitted to Rainbow Babies and Childrens Hospital between 1978-1989 who had discharge diagnoses of encephalitis, were reviewed. Seventy-five met the case definition of presumed viral encephalitis, with viral etiology established in 23% of patients. Poor short-term outcome was defined as the presence of an abnormal neurologic examination at hospital discharge, and was present in 32% of patients. Focal signs on neurologic examination (odds risk: 16.30, P < .05) and abnormal neuroimaging studies (odds risk: 5.66, P < .05) were the only 2 factors present at admission that predicted a poor short-term outcome. Glasgow coma scale at admission was predictive of an abnormal neurologic examination at discharge only when profoundly depressed (6 or less); otherwise, this scale was not useful as a prognostic tool. Factors that were not correlated with adverse outcomes included age younger than 1 year, any type of seizure occurrence, status epilepticus, diffuse or focal electroencephalographic abnormalities, or abnormal cerebrospinal fluid findings.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗

[Dementia evaluated by means of a mini-mental status examination. Clinical neurologic patient material].

The mini-mental state examination (MMSE) was administered to a neurological patient material with various disorders. The test was found useful for detecting cognitive impairment. The test did not differentiate reliably between different causes of cognitive impairment. To detect dementia a cut-off score of 25 or less yielded 100% sensitivity and 97% specificity. The validity of the DSM-III-diagnostic criteria for dementia, primary degenerative dementia and multi-infarct dementia was high. The modified ischemic score (MIS) seemed to be of limited value in differentiating primary degenerative dementia from multi-infarct dementia.

Aged↗

Neurologic signs predict periventricular white matter lesions on MRI.

OBJECTIVE: Periventricular white matter disease (PVWD) is associated with abnormalities on tests that involve complex cognitive processes, along with an increased risk of cerebrovascular events which are associated with significant morbidity in older patients. This study investigates whether the neurological examination can predict the presence of PVWD on magnetic resonance imaging (MRI). No prior studies have assessed whether the neurological examination can predict the presence of PVWD on MRI. METHODS: A focused neurological examination was performed on a random selection of patients referred for a MRI of the brain. Staff neuroradiologists who were blinded to the results of the physical examination independently read the MRI scans. The MRI interpretations were divided into four categories based on the degree of PVWD: none, mild, moderate, severe. RESULTS: Twenty-three subjects had some degree of PVWD, while 25 subjects had none. The total number of neurologic signs correlated significantly with the severity of PVWD even when adjusting for the effect of age (rho=0.67, p<0.001). Ninety-one percent of subjects with PVWD had three or more abnormal signs, while 76% of subjects without PVWD had fewer than three. Abnormalities with the three step motor sequencing and horizontal visual tracking tests were maximally predictive of PVWD. One or both of these tests were abnormal in 96% of subjects with PVWD, while 64% of subjects without PVWD had no problems with either test. CONCLUSION: Simple neurologic tests can predict the presence or absence of PVWD on MRI.

Adolescent↗

Value of repeat cranial computed axial tomography scanning in patients with minimal head injury.

BACKGROUND: Patients with minimal head injury (MHI) and a cranial computed axial tomography (CAT) scan positive for the presence of intracranial injury routinely undergo a repeat CAT scan within 24 hours after injury. The value of this repeat cranial CAT scan is unclear in those patients who are neurologically normal or improving. METHODS: A retrospective analysis of all adult patients admitted to a level-1 trauma center with MHI and a positive cranial CAT scan during a 32-month period was performed. The need for neurosurgical intervention after repeat CAT scan in patients with a persistently normal or improved neurological examination was recorded. RESULTS: One hundred fifty-one patients had a persistently normal or improved neurological examination, but none of these patients required neurosurgical intervention after the repeat cranial CAT scan. CONCLUSIONS: A persistently normal or improving neurological examination in a patient with MHI appears to exclude the need for neurosurgical intervention and thus a repeat cranial CAT scan.

Adult↗

Pediatric AIDS: comparative cranial MRI and CT scans.

Fourteen consecutive children (age range: 4 months to 11 years; median: 4 years) with acquired immunodeficiency syndrome (AIDS) were studied prospectively with cranial magnetic resonance imaging (MRI) and unenhanced computed tomography (CT). In 4 children, human immunodeficiency virus infection was transfusion-related, while in 10, transplacental transmission occurred. Twelve children had abnormal neurologic examinations; of these, 10 had developmental delay and 2 were normal by developmental history and neurologic examination. Standardized neuropsychologic tests were given to all children; 5 were in the normal range and 9 demonstrated significant delays in verbal or motor/perceptual development. All children with abnormal neuropsychologic results were developmentally delayed; however, in 2 infants with normal neuropsychologic assessments, developmental delay and abnormal neurologic examinations were documented. Brain parenchymal volume loss (8 patients) and cervical lymphatic hypertrophy (4 patients) were demonstrated equally well by MRI and CT. CT alone demonstrated striatal-thalamic calcification (1 patient), whereas MRI alone demonstrated delayed myelination (1 patient). The extent of focal white matter lesions in 1 patient was demonstrated better by MRI than by CT. No intracranial mass lesions were demonstrated; however, significant correlations were found between peripheral volume loss imaged by either MRI or CT and both verbal and performance scores. In our small series, MRI offered no apparent advantage over CT in evaluating children with AIDS. We suggest that CT alone is sufficiently sensitive in evaluating pediatric AIDS-related brain abnormalities.

Acquired Immunodeficiency Syndrome↗

Can neurobehavioral examination predict the presence of cerebral injury in the very low birth weight infant?

This study examined in a regional cohort of 66 term age very low birth weight infants, the relationship between qualitative magnetic resonance imaging (MRI) measures of cerebral white and gray matter abnormalities and infant neurobehavioral functioning assessed by structured neurological examination. The diagnostic utility of the Dubowitz neonatal neurological examination in identifying children with severe cerebral abnormalities was also evaluated. Examination results revealed the presence of high rates of neurological abnormality, with 60% of infants scoring in the suboptimal range relative to infants born full term. Linear associations were found between the severity of structural cerebral abnormality on MRI and the quality of clinically rated infant neurobehavioral functioning, with increasing abnormalities being significantly associated with poorer neurological functioning. In particular, white matter abnormalities were significantly associated with lower mean tone and tone pattern scores and a tendency toward lower mean reflex scores. Gray matter abnormalities were significantly associated with lower tone and tone pattern scores and a tendency toward lower spontaneous movements and orientation/behavior scores. Finally, the Dubowitz Neonatal Neurological Examination was found to have relatively good sensitivity (88%; negative predictive value, 92%) but poor specificity (46%; positive predictive value, 34%) for identifying children with significant MRI abnormalities. Implications of these findings for the neurological evaluation of the very low birth weight infant are discussed.

Brain↗

The influence and reliability of neurologic assessment and Karnofsky performance score on prognosis.

The neurologic examination is important in the early diagnosis of brain tumors in children. Only in brain stem gliomas may the neurologic examination be better than computed tomographic scans in determining the progression. However, in general, the traditional neurologic examination has little or no value for prognosis. Reversible, associated features of brain tumors such as seizures or increased intracranial pressure may alter the patient's ability to function neurologically, but may not influence the prognosis regarding the tumor status. The Karnofsky functional status, to a large extent, reflects an adult's ability to work and has prognostic value but is largely inapplicable to children. Thus, a quality-of-life scale for children is needed.

Adult↗

[Electroencephalographic changes in patients with congenital adrenal hyperplasia due to 21-hydroxylase deficiency].

Electroencephalographic changes have been detected in children with premature pubarche. These findings might be the result from past or current central nervous system dysfunction, or from increased sex steroids. In order to test the last assertion we have performed electroencephalographic studies in 10 patients (7F, 3M) with congenital adrenal hyperplasia (CAH) due to 21-hydroxylase deficiency, of whom 4 classic and 6 nonclassic forms of the syndrome. Traditional neurologic examination was performed in 7 patients, the result being considered normal in everyone, while evolutional neurologic examination detected hyperactive disturbance and attention deficit in two patients. In another patient, the clinical findings were compatible with delay of neuropsychomotor development. Quantitative electroencephalography with brain mapping in patients over three years-old (n = 9), and conventional EEG in the remaining patient, were considered abnormal for age in 8 patients (80%), the record being characterized as slow in 7 patients. The high rate of electroencephalographic changes in this sample, as well as in premature pubarche, suggests that these findings might result from high inappropriate for age androgenic levels, and warns about the importance of neurologic examination and follow-up in children with CAH.

Adolescent↗

Treatment of spinal involvement in neuroblastoma patients.

INTRODUCTION: Considerable controversy exists regarding the appropriate management of spinal involvement in neuroblastoma (NB) patients. We review a large group of such patients and offer treatment recommendations. METHODS: Forty-six patients with epidural and/or neural foraminal involvement treated between 1987 and 1998 were staged according to the International NB Staging System (INSS) and classified as high-risk (INSS stage 4; n = 31) or low-risk (INSS stage <4; n = 15). Of 13 high- risk patients with normal neurologic examinations and no radiographic high-grade spinal cord compression (HGSCC), 12 were treated initially with chemotherapy, and only 1 demonstrated neurologic deterioration. HGSCC was present in 18 patients with high-risk NB; 7 of 10 (70%) treated initially with chemotherapy and 6 of 6 (100%) managed initially with operation improved or remained stable. All 9 low-risk patients with normal neurologic examinations and no HGSCC remained neurologically intact following operations (n = 7) or chemotherapy (n = 2). All 4 low-risk patients with HGSCC treated with operations improved or remained stable, and 0 of 2 (0%) low-risk patients treated initially with chemotherapy remained stable. Spinal deformities occurred in 2 of 16 patients (12.5%) treated nonoperatively and in 9 of 30 (30.0%) who underwent operations. CONCLUSIONS: High-risk NB patients with spinal involvement but normal neurologic examinations should be offered chemotherapy. High-risk patients with HGSCC may respond to chemotherapy, but a small percentage will require operations for progressive neurologic deficits. Chemotherapy may be avoided in low-risk patients who are offered potentially curative operations. Patients treated with operations for epidural disease are at high risk of subsequently developing spinal deformity.

Adolescent↗

[Prognosis of perinatal asphyxia in full-term neonates].

We have performed a prospective study in 65 asphyxiated term newborns to evaluate the prognostic value of three different methods: 1) Neurological examination, 2) Electroencephalographic tracings (EEGs) and 3) Transfontanellar sonography, carried out periodically from birth until discharged from the neonatal unit. The neurological status of surviving infants was assessed at one year of age. We found a statistically significant (p < 0.0001) association between the neurological examination, electroencephalographic tracings and transfontanellar sonography during the neonatal period and the neurological development. The prognostic value, as a function of sensibility, specificity and predictive value of the three methods was very high. No differences were found among them, although the EEGs and neurological examinations showed the highest predictive values.

Asphyxia Neonatorum↗

Predicting outcomes of neonates diagnosed with hypoxemic-ischemic encephalopathy.

OBJECTIVE: The goals were to identify predictor variables and to develop scoring systems and classification trees to predict death/disability or death in infants with hypoxic-ischemic encephalopathy. METHODS: Secondary analysis of data from the multicenter, randomized, controlled, National Institute of Child Health and Human Development Neonatal Research Network trial of hypothermia in hypoxic-ischemic encephalopathy was performed. Data for 205 neonates diagnosed as having hypoxic-ischemic encephalopathy were studied. Logistic regression analysis was performed by using clinical and laboratory variables available within 6 hours of birth, with death or moderate/severe disability at 18 to 22 months or death as the outcomes. By using the identified variables and odds ratios, scoring systems to predict death/disability or death were developed, weighting each predictor in proportion to its odds ratio. In addition, classification and regression tree analysis was performed, with recursive partitioning and automatic selection of optimal cutoff points for variables. Correct classification rates for the scoring systems, classification and regression tree models, and early neurologic examination were compared. RESULTS: Correct classification rates were 78% for death/disability and 71% for death with the scoring systems, 80% and 77%, respectively, with the classification and regression tree models, and 67% and 73% with severe encephalopathy in early neurologic examination. Correct classification rates were similar in the hypothermia and control groups. CONCLUSIONS: Among neonates diagnosed as having hypoxic-ischemic encephalopathy, the classification and regression tree model, but not the scoring system, was superior to early neurologic examination in predicting death/disability. The 3 models were comparable in predicting death. Only a few components of the early neurologic examination were associated with poor outcomes. These scoring systems and classification trees, if validated, may help in assessments of prognosis and may prove useful for risk-stratification of infants with hypoxic-ischemic encephalopathy for clinical trials.

Algorithms↗

Bladder functional changes resulting from lipomyelomeningocele repair.

From 1986 to 1991, 12 boys and 23 girls underwent surgery for lipomyelomeningocele removal. Of these patients 29 were 15 months old or younger (average age 3 months), while 6 were 4.5 to 19 years old (average age 10 years). Preoperative and postoperative urodynamic studies, including external urethral sphincter electromyography, were done on everyone. All 29 infants had a cutaneous lesion overlying the lower back and 14 had an abnormal neurological examination. Preoperative urodynamic studies were abnormal in 11 patients, consisting of an upper motor neuron lesion in 6, and a mixed upper and lower motor neuron lesion in 5. Postoperatively, 10 of 14 children with an abnormal neurological examination improved, while 9 of 11 with abnormal lower urinary tract function normalized. In 1 of 18 children (6%) with normal preoperative urodynamic studies detrusor-sphincter dyssynergia developed postoperatively. In all 6 older children urinary incontinence developed, and this led to the diagnosis. Everyone had an abnormal neurological examination and abnormal preoperative urodynamic studies. One child had a lower motor neuron lesion, and 5 had a mixed upper and lower motor neuron lesion. Postoperatively, the neurological examination improved in only 1 patient (16%), and the urological symptoms and urodynamic findings improved in another child. Lipomyelomeningocele has a progressive effect on lower spinal cord function because infants tend to present with fewer urinary manifestations and physical findings than older children. Individuals who escape early detection tend to have a more subtle cutaneous abnormality. As a result, older children are more likely to present with urological and neurological complaints. Surgical correction in infancy provides a degree of reversibility not seen in older children. It is imperative that early identification, evaluation and treatment be undertaken to prevent this progression and permanency of neurological changes and urinary dysfunction.

Adolescent↗

Sensorimotor performance in school-age children with autism, developmental language disorder, or low IQ.

The purpose of the study was to determine the prevalence of 'soft' motor deficits in school-aged children with either developmental language disorder (DLD), autism (with high IQ [HiAD] or low IQ [LoAD]), or low IQ without autism (LoIQ), and to evaluate the utility of a refined neurological examination to discriminate between these groups. A total of 242 children (74% male), aged 7 or 9 years, were evaluated as part of a longitudinal, multi-institutional study, with a standardized neurological examination that included Denckla's Physical and Neurological Examination for Soft Signs. Most of the scores separated children into two groups defined by nonverbal IQ, with the DLD and HiAD groups performing better than the LoAD and LoIQ groups. Exceptions included motor impersistence and stereotypies, which were more likely in the autistic groups. The neurologists' summary clinical impressions indicated better sensory/motor skills, oromotor skills, and praxis in the HiAD than in the DLD children. Inability/unwillingness to perform tasks was much more frequent in LoAD than LoIQ children.

Autistic Disorder↗

Deferral of the rectal examination in blunt trauma patients: a clinical decision rule.

OBJECTIVES: To develop a clinical decision rule that would allow for the safe deferral of the digital rectal examination (DRE) in blunt trauma patients. METHODS: The authors reviewed the medical records of all adult blunt trauma patients meeting trauma team activation criteria over a 14-month period. The results of the DRE and six predictor variables-abnormal neurologic examination, abdominal tenderness, pelvic stability, blood at the urethral meatus, blood pressure < 90 mm Hg, and age over 65 years-were recorded. Patients with abnormal DREs had their discharge summaries reviewed for specific criteria to determine if the abnormal DRE was a true- or false-positive examination. Predictor variables were entered into a classification and regression tree (CART) analysis designed to predict true-positive abnormal DREs. RESULTS: Of the 579 patients, 53 had abnormal DREs, 34 of which were true positives. CART analysis retained three predictors, abnormal neurologic examination, blood at the urethral meatus, and age over 65 years, and accurately classified all patients with a true-positive abnormal DRE. The probability of a true-positive abnormal DRE in a patient with a normal neurologic examination, no blood at the urethral meatus, and age less than 65 years is between 0% and 0.8%. CONCLUSIONS: Adult patients with blunt trauma and a normal neurologic examination, with no blood at the urethral meatus, and who are less than 65 years old have an exceedingly low likelihood of a true-positive abnormal DRE. If validated, patients who meet these three criteria may have the DRE safely deferred.

Abdominal Injuries↗

Linkage analysis and disease models in benign familial infantile seizures: a study of 16 families.

PURPOSE: Benign familial infantile seizures (BFIS) is a genetically heterogeneous condition characterized by partial seizures, onset age from 3 to 9 months, and favorable outcome. BFIS loci were identified on chromosomes 19q12-13.1 and 16p12-q12, allelic to infantile convulsions and choreathetosis. The identification of SCN2A mutations in families with only infantile seizures indicated that BFNIS and BFIS may show overlapping clinical features. Infantile seizures also were in a family with familial hemiplegic migraine and mutations in the ATP1A2 gene. We have examined the heterogeneous genetics of BFIS by means of linkage analysis. METHODS: Sixteen families were examined. Probands underwent neurologic examination, at least one EEG recording, and, when possible, brain CT and MRI. Clinical information about relatives was collected. Families with SCN2A or ATP1A2 mutations were excluded from the study. Chromosome 16p and 19q loci were examined by linkage analysis using two models that differed in penetrance rate. Genetic heterogeneity was evaluated with both models. RESULTS: Clinical information was available for 124 members of affected families. BFIS was diagnosed in 69 subjects. One patient without BFIS had a single febrile seizure, and another had rare episodes of paroxysmal dystonia. Evidence of linkage was obtained only for chromosome 16. Moreover, the high penetrance allowed the identification of genetic heterogeneity. CONCLUSIONS: Our data confirm the relevance of the chromosome 16 locus in BFIS and suggest the presence of an additional locus. This study shows that the genetic model used affects the outcome of linkage analysis.

Brain↗

Neurologic disorders of neonatal foals.

Neurologic examination of the neonatal foal is quite different from the process used to examine older foals and adult horses. Abnormal neurologic signs are best appreciated in the context of a detailed knowledge of general neonatal medicine and awareness of nor-mal foal behavior and milestones of development. A systematic approach to neurologic examination is provided. The results of such examination are used to localize a lesion or lesions in the nervous system. Descriptions and treatment strategies are given for most common and important neonatal neurologic diseases.

Animals↗

Prolongation of the P-300 latency associated with hydrogen sulfide exposure.

Three patients exposed to hydrogen sulfide developed persistent cognitive impairment, as suggested by the P-300 event-related potential and measured by neuropsychological testing. Routine neurological and physical examinations were unremarkable, although the patients were sufficiently impaired so as to be unable to work. The P-300 event-related potential and neuropsychological testing proved to be important in the detection of cognitive dysfunction following acute hydrogen sulfide exposure. The three patients with neurocognitive dysfunction were acutely exposed to hydrogen sulfide. The incidents occurred independently and under different circumstances. Each patient was evaluated at the Northern California Occupational Health Clinic, San Francisco, examined neurologically and neuropsychologically, and evaluated with a P-300 event-related potential. Each patient had persistent neurological symptoms, neuropsychological deficit, and abnormally prolonged P-300 latencies.

Accidents, Occupational↗

Late onset cerebellar degeneration in a middle-aged cat.

Cerebellar degeneration (abiotrophy) (CD) is a spontaneous and accelerated degeneration of one or several mature cerebellar neuronal cell populations and has been described in many domestic animals, especially in dogs, with numerous breed-related cases. In cats, CD is mentioned as a rare sporadic entity. Late onset CDs are exceptionally uncommon and only two cases are reported in young adults, both aged 18 months. This report describes clinical and pathological findings of a late onset feline CD in a 9-year-old male Persian cat. The cat was presented with a history of progressive ataxia lasting 2 years. Neurological examination revealed severe neurological deficits such as generalised and severe ataxia, hypermetria in all four limbs, and bilateral absence of menace response. The lesion was diffusely localised in cerebellum. On gross pathology, the cerebellum appeared of normal size and shape and kidneys were characterised by mild hyperaemia. Histologically, lesions were limited to the cerebellum and kidneys. In the cerebellum, all cerebellar folia of both hemispheres and the vermis were affected. Changes were characterised by severe and diffuse loss of Purkinje cells, loss of cellularity in the granular layer, mild astrogliosis associated with moderate hypertrophy of Bergmann's glia. Immunohistochemistry for feline parvovirus antigen revealed a negative result. Renal lesions consisted of chronic fibrosis associated with chronic interstitial nephritis. CD is a rare disease and occurs commonly in puppies or young animals, who are clinically normal at birth and usually develop neurological signs within a few weeks or months after birth. This report represents the first case of CD in a middle-aged cat.

Animals↗