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EyeSim--an ophthalmic response simulator.

This paper pertains to the design, construction and development of an ophthalmic response simulator, intended to augment current neurological examination teaching practices in medical schools. Neurological examination of the eyes informs a practitioner of the functional state of certain cranial nerves (II, III, IV and VI) through the assessment of visual acuity, visual fields, ocular movements, external and internal eye structures and ophthalmoscopic examination. For the medical student, the interpretation of these indicators may be difficult, compounded by the infrequence with which such patients may be presented. As such, the need was identified for a simulator capable of replicating the ophthalmic response of patients with typical and a range of atypical neurologies. Such a simulator could be used as a stand-alone device or developed toward inclusion in pre-existing, life-size, hi-fidelity mannequins. Specifically, EyeSim will be able to track the movements of a pen-torch and vary the apparent dilation of an iris in response to changing levels of illumination. These responses can then be modulated to reflect certain deficits that neural pathologies may cause. At present the simulator comprises the electrical, electronic and mechanical components that replicate the pupillary response of the iris and the motility of the ocular muscles, a computer for the reception and analysis of a video signal and the micro-controller components, which determine the action of the physical components based on an array of inputs.

Computer Simulation↗

Magnetic resonance imaging in amateur boxers.

Nine amateur boxers who participated in the 1985 and 1986 New York City Golden Gloves competition underwent detailed neurologic examinations and magnetic resonance (MR) scanning. All nine boxers were medically suspended secondary to a knockout or excessive head blows. Neurologic examination results and MR scans were normal in all nine boxers. Failure to detect abnormalities on the MR scan, by neurologic examination, or both in these amateur boxers may reflect several factors, including a small sample size, the duration between their last bout and neurologic evaluation, and the lower exposure to head trauma among amateur boxers compared with professionals.

Adolescent↗

[A 78-year-old woman who had an onset of seizure and right hemiparesis at the age 77].

We report a 78-year-old woman who had an onset of convulsion and right hemiparesis at the age 77. She had been well until October 28th of 1990 when she suddenly developed a seizure starting in her right face with secondary generalization. She was admitted to Saitama Kyodo Hospital where neurologic examination revealed confusion with slight right hemiparesis; deep reflexes were exaggerated on the right side; otherwise neurologic examination was unremarkable. Cranial CT scan revealed an iso-density mass in the left motor area with extensive edema extending into left anterior frontal as well as parietal regions; by contrast enhancement, a homogeneous enhancement of the tumor was noted. She was treated with glycerol and phenytoin, and she became alert two days after her admission. The diagnosis of metastatic brain tumor was entertained; extensive malignancy survey was performed, however, no primary tumor was found. As neurosurgical procedure was refused, she was discharged on December 16th of 1990. She noted worsening of her right hemiparesis in the end of February, 1991, and she was admitted again on March 18th of 1991. On neurologic examination, she was disoriented to time and place; she was apparently demented. Her right hemiparesis was more advanced and she was unable to walk. Her hospital course was complicated by disturbance of consciousness and pneumonia, and she died on August 22nd of 1991. The patient was discussed in a neurological CPC. Opinions were divided between meningioma and a metastatic brain tumor. Other possibilities raised included malignant lymphoma and glioblastoma multiforme. As edema was very extensive on CT, many participants thought that it might be a metastatic brain tumor.(ABSTRACT TRUNCATED AT 250 WORDS)

Aged↗

Neuroimaging in the evaluation of patients with non-acute headache.

Available studies offer only limited guidance on neuroimaging of non-acute headache patients. The aim of this study was to estimate the frequency of significant intracranial lesions in patients with headache and to determine the clinical variables helpful in identifying patients with intracranial lesions. All patients aged >or= 15 years attending the Neurology Clinic with non-acute headache were included in the study and followed prospectively. Every patient was investigated by neuroimaging studies, either computed tomography or magnetic resonance imaging. Neuroimaging results were classified as 'significant abnormalities', 'non-significant abnormalities' or 'normal'. Significant abnormalities included neoplastic disease, hydrocephalus, vascular malformations, Chiari malformation, large arachnoid cysts, intracranial haemorrhage, and acute cerebral infarcts. Consecutive patients (n=1876; 1243 women and 633 men) were included. Their mean age was 38 years (range 15-95 years). Neuroimaging studies detected significant lesions in 22 patients [1.2%, 95% confidence interval (CI) 0.7, 1.8]. The rate of significant intracranial abnormalities in patients with headache and normal neurological examination was 0.9% (95% CI 0.5, 1.4). The only clinical variable associated with a higher probability of intracranial abnormalities was neurological examination. The proportion of patients with headache and intracranial lesions is relatively small, but neither neurological examination nor the features in the clinical history permit us to rule out such abnormalities.

Adolescent↗

Practice parameter: evaluation of children and adolescents with recurrent headaches: report of the Quality Standards Subcommittee of the American Academy of Neurology and the Practice Committee of the Child Neurology Society.

OBJECTIVE: The Quality Standards Subcommittee of the American Academy of Neurology and the Practice Committee of the Child Neurology Society develop practice parameters as strategies for patient management based on analysis of evidence. For this parameter, the authors reviewed available evidence on the evaluation of the child with recurrent headaches and made recommendations based on this evidence. METHODS: Relevant literature was reviewed, abstracted, and classified. Recommendations were based on a four-tiered scheme of evidence classification. RESULTS: There is inadequate documentation in the literature to support any recommendation as to the appropriateness of routine laboratory studies or performance of lumbar puncture. EEG is not recommended in the routine evaluation, as it is unlikely to define or determine an etiology or distinguish migraine from other types of headaches. In those children undergoing evaluation for recurrent headache found to have a paroxysmal EEG, the risk for future seizures is negligible; therefore, further investigation for epilepsy or treatments aimed at preventing future seizures is not indicated. Obtaining a neuroimaging study on a routine basis is not indicated in children with recurrent headaches and a normal neurologic examination. Neuroimaging should be considered in children with an abnormal neurologic examination or other physical findings that suggest CNS disease. Variables that predicted the presence of a space-occupying lesion included 1) headache of less than 1-month duration; 2) absence of family history of migraine; 3) abnormal neurologic findings on examination; 4) gait abnormalities; and 5) occurrence of seizures. CONCLUSIONS: Recurrent headaches occur commonly in children and are diagnosed on a clinical basis rather than by any testing. The routine use of any diagnostic studies is not indicated when the clinical history has no associated risk factors and the child's examination is normal.

Adolescent↗

The effects of delayed diagnosis and treatment in patients with an occult spinal dysraphism.

From 1987 to 1993, 21 older individuals presented for the first time with signs and symptoms that eventually led to the diagnosis of occult spinal dysraphism. Assessment consisted of a neurological examination, urodynamic studies preoperatively and postoperatively, and spinal cord imaging. Of 21 patients 18 had an abnormal neurological examination, whereas only 15 had an abnormal urodynamic study, as judged by sphincter electromyography. Radiological imaging showed that 9 patients had a tethered cord alone, 4 each had lipomeningocele and lipoma, 2 had a bony spine abnormality and 1 each had thoracic meningocele and diastematomyelia. Of the 21 patients 19 underwent spinal surgery. Postoperatively, the neurological examination improved in 1 case (5%) and remained unchanged in 18 (95%), while urodynamic findings improved in 3 (16%), were unchanged in 11 (68%) and worsened in 5 (26%). Six patients had progressive deterioration and required secondary spinal surgery, which helped only 2 (33%). These observations confirm that older children and adults with occult spinal dysraphism are more likely to present with irreversible urological and neurological findings than younger children, and so it is imperative that a diagnosis be made and treatment be instituted as early as possible.

Adult↗

Quality of general movements and the development of minor neurological dysfunction at toddler and school age.

OBJECTIVE: To evaluate the reliability of assessing infants' general movements (GMs) using a new classification and its validity in predicting complex minor neurological dysfunction (MND) at toddler and at school age. DESIGN: Prospective study of two groups of infants, each consisting of a mix of low-risk and high-risk infants. SETTING: University Hospital Groningen, the Netherlands. SUBJECTS: Group A consisted of 16 low-risk and 21 high-risk infants; group B of 28 low-risk and 24 high-risk infants. MAIN OUTCOME MEASURES: Between term age and four months post term: multiple assessments of neurological condition by means of (a) assessment of GMs, and (b) a traditional neurological examination. GMs were classified into four classes using a standardized qualitative description: two classes of normal movements (normal-optimal and normal-suboptimal) and two classes of abnormal movements (mildly and definitely abnormal movements). Follow-up neurological examination with special attention to presence of MND was carried out in group A at 1 1/2 years, in group B at 4-9 years. RESULTS: GMs could be assessed reliably. They were stable over age in about 60% of the infants. Both the condition of the GMs and the infant neurological condition were significantly related to neurological condition at follow-up. Best prediction of complex MND was achieved when both types of infant assessment at the age of 2-4 months post term were combined. CONCLUSIONS: The assessment of GMs is a valuable tool, in particular when combined with the traditional neurological examination, to predict at early age the development of complex MND.

Child↗

Psychiatric symptoms do not correlate with cognitive decline, motor symptoms, or CAG repeat length in Huntington's disease.

OBJECTIVES: To investigate the hypothesis that psychiatric disturbances in Huntington's disease are related to degree of cognitive or motor compromise and to determine correlations between CAG repeat length within the gene for Huntington's disease and disease severity. DESIGN: Consecutive series of patients with Huntington's disease. SETTING: Neurological specialty hospital. PATIENTS: Seventeen men and 12 women from 24 families. MAIN OUTCOME MEASURES: The Hamilton Psychiatric and Anxiety Rating Scales and Brief Psychiatric Rating Scale were used to assess psychiatric disturbances; Folstein's Quantified Neurological Examination to evaluate motor status; and the Mini-Mental State Examination, Raven Progressive Matrices), Phonemic Verbal Fluency Test, Short Tale Test, Visual Search Test, and Benton's Visual Orientation Line Test to evaluate cognitive function. The length of the CAG repeat sequence in the Huntington's gene was determined by quantitative polymerase chain reaction. RESULTS: Cognitive test scores correlated significantly with each other; of these, results of the Visual Search and Short Tale tests correlated significantly with the Folstein's Quantified Neurological Examination score (P = .05 and P = .03, respectively). Results of the Folstein's Quantified Neurological Examination also correlated with the illness duration and the length of the CAG repeat. Although psychiatric scores correlated significantly among themselves (P < .01), neither cognitive compromise, motor deterioration, nor CAG length were related to the extent of psychiatric compromise. Patients who were depressed when they were examined tended to have a history of psychiatric disorders. CONCLUSIONS: The lack of correlation between disease severity and psychiatric disturbances indicates that psychiatric disorders progress nonlinearly, possibly because of differential degeneration of the striatal-cortical circuits; the possibility that psychiatric disorders are prevalent in certain families with a member who has Huntington's disease is being further investigated. The lack of correlation between CAG length and cognitive and psychiatric variables needs further investigation.

Adult↗

Prediction of intracranial metastases in cancer patients with headache.

BACKGROUND: The current study was conducted to investigate the diagnostic value of neurologic evaluation for the prediction of intracranial metastases in cancer patients with new or changed headache. METHODS: Between February 1997 and February 2000, general practitioners and specialists referred cancer patients with new or changed headache to the Department of Neurology at the study institution. All patients underwent a structured history and neurologic examination. Magnetic resonance imaging (MRI) of the brain was used as the gold standard for determining the presence of intracranial metastases. The association between baseline patient characteristics, history variables, and variables from the neurologic examination in patients with intracranial metastases was evaluated by univariate and multivariate logistic regression analyses in combination with receiver operating characteristic (ROC) curve analyses. RESULTS: Sixty-eight consecutive patients were included in the current study (48 females and 20 males). The mean age of the patients was 57 years (range, 24-88 years; standard deviation +/- 13.3 years). Breast carcinoma was the primary tumor in 32 patients (47.1%) and lung carcinoma was the primary tumor in 12 patients (17.6%). Intracranial metastases occurred in 22 patients (32.4%). The occurrence of intracranial metastases was predicted in the multivariate logistic regression analyses by one baseline patient characteristic variable and 2 history variables (i.e., headache duration of < or =10 weeks [odds ratio (OR) of 11.0; 95% confidence interval (95% CI), 1.1-108.2], emesis [OR of 4.0; 95% CI, 1.1-14.3], and pain not of tension- type [OR of 6.7; 95% CI, 1.8-25.1]). No variable from the neurologic examination was found to add information to the prediction model. When at least one of the three predictors was present, all patients with intracranial metastases could be identified with this prediction model. MRI could be omitted in 12 patients (26%) without intracranial metastases. The ROC area under curve of this model was 0.83. CONCLUSIONS: Intracranial metastases were found in 32.4% of the cancer patients with headache as the presenting symptom. Although 3 significant clinical predictors were found (headache duration < or =10 weeks, emesis, and pain not of tension- type), few patients could be excluded from undergoing MRI because of a low specificity. Therefore, MRI of the brain was considered to be warranted in all patients in the current study.

Adult↗

Sensory exam with a quantitative tuning fork: rapid, sensitive and predictive of SNAP amplitude.

BACKGROUND: In the standard neurologic examination, outcome measures of sensation testing are typically qualitative and subjective. The authors compared the outcome of vibratory sense evaluation using a quantitative Rydel-Seiffer 64 Hz tuning fork with qualitative vibration testing, and two other features of the neurologic evaluation, deep tendon reflexes and sensory nerve conduction studies. METHODS: The authors studied 184 subjects, including 126 with Waldenström's macroglobulinemia and 58 controls, over the course of a weekend. Standard neurologic examinations and quantitative vibratory testing were performed. Sensory nerve action potentials (SNAP) were tested as a measure of sensory nerve function. Tests were carried out by different examiners who were blinded to the results of other testing and to clinical information other than the diagnosis of Waldenström's macroglobulinemia. RESULTS: Quantitative vibration measurements in all body regions correlated with sural SNAP amplitudes. Quantitative vibration outcomes were more strongly related to sural SNAP results than qualitative evaluations of vibration. Quantitative vibration testing also detected a loss of sensation with increased age in all body regions tested. CONCLUSIONS: Quantitative vibratory evaluation with Rydel-Seiffer tuning fork is rapid, has high inter- and intrarater reliability, and provides measures for evaluating changes in sensory function over time. Examinations with the quantitative tuning fork are also more sensitive and specific than qualitative vibration testing for detecting changes in sensory nerve function. Use of the quantitative tuning fork takes no more time, provides more objective information, and should replace the qualitative vibratory testing method that is now commonly used in the standard neurologic examination.

Action Potentials↗

Does status epilepticus in children cause developmental deterioration and exacerbation of epilepsy?

The aims of this study were to determine predictors of abnormal outcome, neurodevelopmental deterioration, new-onset epilepsy, refractory epilepsy, and recurrent status epilepticus in children presenting with status epilepticus. For all children presenting to Royal University Hospital, Saskatoon, Saskatchewan, Canada, with status epilepticus between January 1987 and December 1996, demographic data, details of status epilepticus (etiology, duration, treatment, and investigations), developmental milestones, seizures prior to and following status epilepticus, recurrent status epilepticus, and neurologic examination findings at status epilepticus and at follow-up were collected by chart review, patient interview, and neurologic examination. Neurodevelopmental outcome was determined for all subjects except those who died during the initial hospitalization. Predictors of new-onset epilepsy, refractory epilepsy, and recurrent status epilepticus were determined for children followed for 3 months or more after status epilepticus. At follow-up, 79% were abnormal neurologically. Predictors included etiology (nonfebrile or nonidiopathic), perinatal difficulties, preceding developmental delay, abnormal initial neurologic examination; and abnormal neuroimaging. Thirty-four percent showed neurodevelopmental deterioration; predictors included etiology (nonidiopathic or nonfebrile), young age at status epilepticus (12 months or less), and abnormal neuroimaging. Thirty-six percent with no history of seizures preceding status epilepticus developed epilepsy and 25% developed refractory epilepsy. Fifty percent of children had recurrent status epilepticus. In conclusion, very few children presenting in status epilepticus were normal at follow-up. Sequelae were seen predominantly in those with a nonidiopathic, nonfebrile etiology, whereas those with idiopathic or febrile status epilepticus did well.

Adolescent↗

Neurological status in severely jaundiced Zimbabwean neonates.

Neurological status was studied in 50 jaundiced infants with a total serum bilirubin of > 400 mumol/l (23.4 mg/dl). Infants were assessed in the neonatal period with the Neonatal Neurological Examination and 4 months of age with the Infant Motor Screen. Twenty-six (52 per cent) infants were premature. Analysis of variance did not show a significant difference between gestational age, birth weight, and maximum total serum bilirubin or between gestational age, birth weight, and neurological optimality score. Based on the presence of abnormal neurological syndromes the infants were classified as normal (n = 27), suspect (n = 11), or abnormal (n = 12). Serum bilirubin levels were higher (p < 0.0001) and the neonatal neurological examination scores lower (p < 0.0001) in the seven (14 per cent) infants who received an exchange transfusion. In the transfused group four out of seven infants and in the non-transfused group seven out of 43 infants were classified as abnormal (p < 0.03). The Neonatal Neurological Examination was shown to be sensitive in detecting neurodevelopmental abnormalities in the neonatal period, with a sensitivity of 83 per cent, specificity of 88 per cent, positive predictive value of 62 per cent, and negative predictive value of 96 per cent.

Analysis of Variance↗

Detrusor hyperreflexia. A survey on its etiology and treatment.

Detrusor hyperreflexia (DH) is a frequently occurring condition. The symptomatology is characterized by frequency, urgency and urge incontinence. DH is defined as involuntary, uninhibited detrusor contractions. The physiology and pathophysiology of the micturition reflex is reviewed. The balance between cerebral stimulation and supraspinal inhibition is discussed. DH is caused by disturbances in this balance. Whereas increased afferent impulses to the central nervous system due to local disorders in bladder and/or urethra may produce DH, a neurological disorder affecting the inhibitory nervous pathways from cortical and subcortical centres always result in uninhibited detrusor contractions. DH was found in 25% of 2000 patients. In the majority of the patients the DH was caused by a neurological disorder. The incidence of DH in patients with enuresis, gynecological patients with urinary incontinence and/or genital prolapse and patients with benign prostatic hyperplasia (BPH) is reported. In 62% of the patients with BPH the DH was eliminated after adequate surgical treatment of the infravesical obstruction. By contrast, DH in women with genital prolapse and/or incontinence persisted despite operative treatment. In a retrospective investigation of 152 patients with DH, the cause of the DH was unknown in 32 patients (21%). A clinical neurological examination revealed no evidence of neurological disease in 45% of the 22 patients examined. Voiding symptoms were the only complaint in these patients as well as in 30-40% of the patients in the other groups mentioned. This calls for improved investigatory methods in the evaluation of the balance between stimulation and inhibition of the micturition reflex. The presence of uninhibited detrusor contractions in apparently healthy patients should indicate a neurological examination since DH may be the first sign of a neurological disorder. The micturition reflex is conducted through long, uninterrupted neurons with a marked central integration. Therefore cystometry may be used as a supplement to the clinical neurological examination in the early diagnosis of pyramidal or extrapyramidal central nervous system disorders.

Adolescent↗

Tolerance of initial diphtheria-tetanus-pertussis immunization in preterm infants.

With the use of neurologic examinations, cranial sonograms, electroencephalograms, an cry analyses, we assessed neurologic function before and after an initial diphtheria-tetanus-pertussis immunization in 22 very low birth weight infants. Mean birth weight was 1036 +/- 137 gm; mean gestational age was 28.0 +/- 1.3 weeks. All 22 infants had recovered from respiratory distress syndrome, 10 infants had been treated for invasive bacterial or fungal infection, and 9 infants had had previous intraventricular hemorrhages. Initial immunization was administered at a mean age of 71 +/- 15 days (range 57 to 120 days) and a mean weight of 1895 +/- 245 gm (1370 to 2280 gm). Clinical reactions were mild and transient. No postimmunization changes in neurologic examinations or objective studies were noted compared with results of preimmunization studies. These findings support the safety of administering an initial diphtheria-tetanus-pertussis immunization to very low birth weight infants at the recommended age of 8 weeks.

Diphtheria-Tetanus-Pertussis Vaccine↗

Obstetrical-neonatal neurological relationship. A replication study.

A study concerning the relationship between neonatal neurological abnormality and 3 parameters of pre- and perinatal condition in a birth cohort of 1507 infants was replicated in a second cohort of 1655 infants. The 3 parameters chosen were preterm birth, intrauterine growth retardation and neonatal acidemia. The neurological examination in the second cohort was carried out by other examiners than in the first cohort. The incidence of neurological abnormality was again found to be raised in all 3 categories and again least in the acidemic subgroup. Also the finding could be confirmed that only in about half of the neurologically abnormal infants could the 3 parameters have exerted any effect at all, which implies the necessity of further analysis. This replication study shows that neurological examination in the newborn period is a reliable tool for the evaluation of the condition of infants with respect to their pre- and perinatal history.

Blood Gas Analysis↗

Subclinical skeletal muscle involvement in long-QT syndrome.

Deafness is said to be the only extracardiac manifestation of long-QT syndrome. Whether long-QT syndrome manifests in the skeletal muscle as well, has not been investigated so far. Six affected members of two families with long-QT syndrome without deafness (Romano-Ward syndrome) underwent a clinical neurological examination, nerve conduction studies and needle electromyography. The clinical neurological examination and nerve conduction studies were normal but abundant spontaneous activity (fibrillations and bursts of fibrillations) could be recorded from the right biceps brachii muscle (one patient) and the right abductor pollicis brevis muscle (all patients). Since all other causes were excluded, spontaneous discharges were interpreted to be related to the long-QT syndrome. In conclusion, long-QT syndrome does not seem to be confined to the heart but may involve the skeletal muscle subclinically as well.

Action Potentials↗

Relation of neurological findings after cardiac arrest to outcome.

Routine neurological examination of patients one hour after cardiac arrest seems to be of value in determining the prognosis for life and likelihood of intellectual impairment.In 48 patients 53 episodes of cardiac arrest were followed by serial neurological examinations. Patients were divided into two groups according to neurological findings one hour after cardiac arrest. Patients in group 1 were unresponsive or at most responded in a reflex fashion to painful stimuli at one hour; these patients died or survived with intellectual damage. Patients in group 2 responded purposefully at one hour and survived without neurological damage. These patients commonly showed transient confusional states and a variety of other non-focal abnormalities, and focal signs were seen occasionally.

Adult↗