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Cramped synchronized general movements in preterm infants as an early marker for cerebral palsy.

OBJECTIVE: To ascertain whether specific abnormalities (ie, cramped synchronized general movements [GMs]) can predict cerebral palsy and the severity of later motor impairment in preterm infants affected by brain lesions. DESIGN: Traditional neurological examination was performed, and GMs were serially videotaped and blindly observed for 84 preterm infants with ultrasound abnormalities from birth until 56 to 60 weeks' postmenstrual age. The developmental course of GM abnormalities was compared with brain ultrasound findings alone and with findings from neurological examination, in relation to the patient's outcome at age 2 to 3 years. RESULTS: Infants with consistent or predominant (33 cases) cramped synchronized GMs developed cerebral palsy. The earlier cramped synchronized GMs were observed, the worse was the neurological outcome. Transient cramped synchronized character GMs (8 cases) were followed by mild cerebral palsy (fidgety movements were absent) or normal development (fidgety movements were present). Consistently normal GMs (13 cases) and poor repertoire GMs (30 cases) either lead to normal outcomes (84%) or cerebral palsy with mild motor impairment (16%). Observation of GMs was 100% sensitive, and the specificity of the cramped synchronized GMs was 92.5% to 100% throughout the age range, which is much higher than the specificity of neurological examination. CONCLUSIONS: Consistent and predominant cramped synchronized GMs specifically predict cerebral palsy. The earlier this characteristic appears, the worse is the later impairment.

Cerebral Palsy↗

Clinical utility of quantitative cerebral blood flow measurements during internal carotid artery test occlusions.

OBJECTIVE: Internal carotid artery (ICA) balloon test occlusions (BTOs) are performed in the angiography suite to predict whether the patient has adequate collateral circulation to prevent stroke when permanent ICA occlusion (PCO) is required for treatment. Although many criteria have been proposed to facilitate predictions of stroke risk after PCO, no BTO techniques have been subjected to predictive validity testing in outcome studies. We describe a prospective case series study that tests the predictive validity of quantitative cerebral blood flow (CBF) measurements during ICA BTO. METHODS: Thirty-three patients with clinical indications for PCO underwent ICA BTO and then PCO. During BTO, standard neurological examinations, sustained-attention testing, and quantitative CBF measurements were performed. Two scalp scintillation detectors recorded washout data after ipsilateral intracarotid injection of xenon-133 through a port at the tip of the ICA-occluding balloon. Patients were monitored for the outcome measure of ipsilateral stroke for a mean of 34 months. The variables of quantitative CBF values, neurological examination results, sustained-attention test results, age, sex, and side of occlusion were examined with Kaplan-Meier log-rank tests, predictive validity analyses, and logistic regression analyses. RESULTS: CBF of less than 30 ml/100 g/min during BTO was the only variable that predicted stroke after PCO (log rank = 5.87, P = 0.015). The negative and positive predictive values for CBF findings were superior to those for standard neurological examination findings and sustained-attention test results. Age, sex, and side of occlusion did not predict stroke. CONCLUSION: Quantitative CBF testing, via the intracarotid injection technique, during BTO seems to be an important predictor of stroke after PCO.

Adult↗

Neonatal hyperbilirubinemia and long-term outcome: another look at the Collaborative Perinatal Project.

OBJECTIVE: To examine the association between neonatal bilirubin levels and subsequent neurodevelopmental outcome. DESIGN: Prospective cohort study. SETTING: 12 US medical centers from 1959 (first births) to 1974 (last follow-up). PARTICIPANTS: 41,324 singleton white or black infants with birth weight > or = 2500 g who had neonatal bilirubin measurements recorded and survived at least 1 year. MAIN OUTCOME MEASURES: Wechsler Intelligence Scale for Children Intelligence Quotient (IQ) at age 7 years, blinded neurologic examination at age 7 years, and sensorineural hearing loss at age 8 years. RESULTS: There was no association between IQ and bilirubin. For example, comparing children who had maximum bilirubin levels > or = 342 mumol/L (20 mg/dL) with those who had lower bilirubin levels, adjusted mean IQs were 105.0 and 103.4 in whites (difference + 1.6; 95% confidence interval [CI]: -0.4 to +3.5) and 91.0 and 93.3 in blacks (difference -2.3; 95% CI: -4.8 to +0.2). Abnormal neurologic examination results were reported in 12 of 268 children (4.5%) with bilirubin > or = 342 mumol/L (20 mg/dL) compared with 1249 of 33,004 children (3.8%) with lower levels (relative risk [RR] = 1.2; 95% CI: 0.7 to 2.1). The frequency of abnormal or suspicious neurologic examination results increased in a stepwise fashion with increasing bilirubin level (P < .001), from 4346/29,258 (14.9%) of those with bilirubin levels < 171 mumol/L (10 mg/dL) to 60/268 (22.4%) of those with bilirubin levels. > or = 342 mumol/L (20 mg/dL), apparently due to increasing minor motor abnormalities at higher bilirubin levels. Sensorineural hearing loss was not associated with high bilirubin levels (RR = 1.0; 95% CI: 0.3 to 3.0). CONCLUSIONS: Neonatal bilirubin levels seem to have little effect on IQ, definite neurologic abnormalities, or hearing loss. Higher bilirubin levels are associated with minor motor abnormalities, but the clinical importance of this finding is limited by the weakness of the association, the mild nature of the abnormalities, and the lack of evidence that they are prevented by treatment.

Bilirubin↗

[Late neurological complications of heart transplantation].

To assess anomalies in these subjects, an ambulatory neurological examination was performed in 12 heart transplant patients and in 1 heart-lung transplant patient. The patients were examined between the 3rd and 51st month following the transplantation. Two had symptoms due to a previous neurological disease. Early postoperative complications were found in 6 patients (seizures in 3 cases, cerebral infarcts in 1 case, anoxia in 1 case and right ulnar nerve damage at the elbow in 1 case). Late postoperative complications included postural tremor (9 cases) or mild polyneuropathy (2 cases). Neurological examination was completely normal in 2 patients. The findings show that the most common late neurological abnormality found in heart transplant patients is postural tremor.

Adult↗

Objective assessment of cervical spinal cord injury levels by transcranial magnetic motor-evoked potentials.

BACKGROUND: The neurologic examination serves as the optimal method to record the level of spinal cord injury (SCI). However, this test is subject to interexaminer variability. To address this shortcoming, we describe a technique that uses transcranial magnetic motor-evoked potentials (tcMMEPs) and dermatomal somatosensory-evoked potentials (d-SSEPs) to more accurately measure the precise level of SCI. METHODS: Two groups of subjects were studied: (1) complete cervical SCI (n = 10) and (2) neurologically intact volunteers (n = 10). Two additional patients were evaluated: one with a cervical central spinal cord syndrome and another with a head injury with a suspected cervical SCI. Each subject underwent upper extremity tcMMEPs and d-SSEPs. RESULTS: Transcranial magnetic motor-evoked potentials were elicited from all upper limb myotomes (C4-T1, bilaterally) in neurologically intact volunteers (20 sides). The level of injury was determined using tcMMEPs by observing the lowest level of measurable response. The level of injury obtained using tcMMEPs was the same as that determined by neurologic examination in 13 (65%) of the 20 sides. In 7 sides, tcMMEP responses were obtained 1 level lower than that assessed by physical examination. Dermatomal somatosensory-evoked potentials were obtained from all dermatomes of volunteers tested in the laboratory compared with only 5 of the 9 patients with SCI who underwent d-SSEP testing. CONCLUSION: Testing using tcMMEPs provides an objective supplement to the neurologic examination after acute cervical SCI. Dermatomal somatosensory-evoked potentials were of limited value in determining the level of cervical SCI.

Adolescent↗

[Pitfalls in MR imaging of neurological diseases].

MR imaging is useful for the diagnosis of neurological diseases but it has many pitfalls. The author presented one patient with syringobulbia of whom neurological examinations revealed lesions in the medulla. MR images showed slit-like lesions in the medulla and pontine tegmentum of which signal intensity was similar to that of cerebrospinal fluid. However, a sagittal scout image reveals syringomyelia in the upper cervical spinal cord that was important abnormal finding for the diagnosis of this case. In a case of hypertrophic cranial pachymeningitis, neurological examinations showed right lower cranial palsies and MR images showed lesions along the right temporal bone with T1- and T2 shortening. After injection of the contrast medium, abnormal contrast enhancement was observed in the medial side of the lesions. In the anterior cranial fossa, however, the anterior falx was thick and it showed low signal on T2-weighted images and abnormal contrast enhancement. These findings of the falx indicated that the patient had hypertrophic cranial pachymeningitis. It is important to see lesions which neurological examinations detect. However, all of the images must be seen, because in the distant location, the key findings for the diagnosis may be present.

Central Nervous System↗

[Pediatric neuroimaging emergencies].

The notion of emergency with regards to pediatric neuroimaging requires a strong knowledge of clinical indications. In children under 2 years of age, head trauma requires a CT scan in case of repeated or prolonged or rapidly increasing vomiting, focal signs, loss of consciousness, unusual behavior, seizures, clinical signs of skull fracture or polytrauma. The "shaken baby syndrome" is usually suspected in case of loss of consciousness or seizures before 8 months of age. The hematomas that are observed are subdural in location, diffuse and deeply located. Imaging is only mandatory for headache suggesting underlying space occupying lesion: permanent or increasing pain, nocturnal headache, headache during postural changes or efforts, associated to seizures or abnormal neurological examination. No imaging is indicated in case of first epileptic seizure associated to normal neurological examination and without any particular context. The presence of trauma, intracranial hypertension, persisting disturbances of consciousness or associated focal sign necessitates urgent neuroimaging. No imaging is indicated in case of typical febrile seizures, i.e. generalized, brief and occurring between 1 and 5 years of age. Spinal cord symptoms require immediate MRI evaluation. The most frequent tumor is neuroblastoma. In the absence of spinal tumor, brain abnormalities must be excluded (inflammatory disease). In neonates, CT scan or MRI must be readily performed in case of seizures or loss of consciousness to exclude ischemic, traumatic or infectious lesions.

Age Factors↗

Immediate S-100B and neuron-specific enolase plasma measurements for rapid evaluation of primary brain damage in alcohol-intoxicated, minor head-injured patients.

The neuroproteins S-100B and neuron-specific enolase (NSE) released into the circulation are suggested to be reliable markers for primary brain damage. However, safe identification of relevant post-traumatic complications after minor head injury (MHI) is often hampered by acute intoxication of the patients. The objective of this study was to determine the diagnostic validity of immediate plasma measurements of S-100B and NSE in comparison with neurological examinations and cerebral computed tomography (CCT) findings in alcohol-intoxicated MHI patients. One hundered thrity-nine MHI individuals were enrolled in this prospective study during Munich's Oktoberfest 2000. Plasma levels of S-100B and NSE as well as serum alcohol and glucose values were determined by fully automated assays immediately after admission. The results were compared with Glasgow Coma Scale score, a brief neurological examination, and the CCT findings. Without being influenced by alcohol, median S-100B levels of the CCT+ group were significantly increased compared with those of the CCT- group (P < 0.001). NSE, alcohol, and glucose levels showed no significant group differences. As calculated by the ROC analysis, a cutoff value of 0.21 ng/mL with an area under the curve of 0.864 clearly differentiates between CCT+ and CCT- patients at a sensitivity of 100%, a specificity of 50.0%, and a positive likelihood ratio of 2.0. Although acute alcohol intoxication did not confound plasma measurements of S-100B and NSE, only S-100B levels below the cutoff level of 0.21 ng/mL seem to indicate absence of primary brain damage. Thus, in addition to routine neurological examinations, S-100B measurements immediately after admission might help to reduce CCT scans in alcohol-intoxicated patients early after MHI.

Adult↗

Primitive reflexes in a case-control study of patients with advanced human immunodeficiency virus type 1.

This study estimated the frequency of nine primitive reflexes (PR) and assessed their possible clinical value in a group of patients with acquired immunodeficiency syndrome. We studied 78 patients with human inmunodeficiency type 1 (HIV-1) infection in WHO clinical stage 3 or 4 and 81 matched seronegative controls. All participants were examined using a standardized neurological examination and the Mini-Mental State Examination. Cognitive impairment and PR was found in 36% of patients but in none of the controls (P<0.0001; logistic regression odds ratio: 14.7). Overall, PR were 2-36 times more frequent in patients with HIV-1 infection. This association was stronger for the glabellar, snout, Rossolimo, and digital signs. At least two PR were observed in 92% of patients vs. 8% of controls (P<0.0001; 95% confidence interval: 68%-100%; logistic regression odds ratio: 10.8). These data support the association of PR with cognitive decline in patients with advanced HIV-1 infection without overt neurological disease. Larger follow-up studies with multivariate techniques are needed to identify which PRs are useful as indicators of HIV-1-associated cognitive/motor complex and minor neurocognitive disorders.

Acquired Immunodeficiency Syndrome↗

Critical periods of suicide risk in Huntington's disease.

Contrary to popular belief, receiving a diagnosis of a devastating fatal disease does not exacerbate, and may even alleviate, the risk of suicide. Suicidal ideation was examined in 4,171 individuals in the Huntington Study Group database. Participants were grouped according to a standardized neurological examination from 0 (i.e., normal examination) to 3 (definite Huntington's disease). Patients with an unequivocal diagnosis of Huntington's disease were further divided by stage of disease, from stage 1 (early) to stage 5 (end stage). Findings showed that the frequency of suicidal ideation doubled from 9.1% in at-risk persons with a normal neurological examination to 19.8% in at-risk persons with soft neurological signs and increased to 23.5% in persons with "possible Huntington's disease." In persons with a diagnosis of Huntington's disease, 16.7% had suicidal ideation in stage 1, and 21.6% had suicidal ideation in stage 2, whereas the proportion of Huntington's disease patients with suicidal ideation diminished thereafter. Findings suggest two critical periods for increased risk of suicide in Huntington's disease. The first critical period is immediately before receiving a formal diagnosis of Huntington's disease, and the second is in stage 2 of the disease, when independence diminishes. Although the underlying mechanisms of suicidal ideation in Huntington's disease are poorly understood, it is critical for health care providers to be aware of periods during which patients may be at an increased risk.

Adult↗

Lower motor neuron disease associated with myelofibrosis.

We present a patient who has signs pointing to the involvement of lower motor neurons and myelofibrosis. To our knowledge, unlike lymphoproliferative disorders, co-occurrence of myelofibrosis and lower motor neuron disease (MND) has not been reported so far. A 64-year-old male patient was admitted to our hospital with the complaint of painful cramps in his neck and forearms. On physical examination marked hepatomegaly and splenomegaly were found. On neurologic examination nasal quality of the voice and slight palatal weakness were detected. There were generalised slight weakness and atrophy in both proximal and distal muscle groups. Fasciculations were observed especially in forearm muscles and it was observed that he had been avoiding head movements because of painful muscle cramps. Deep tendon reflexes were hypoactive. Nerve conduction studies were normal. By needle electromyography, giant motor unit action potentials (amplitudes up to 8 mV), fibrillation potentials, positive sharp waves and fasciculation potentials were detected in all muscles which were investigated. A hypercellular bone marrow (100%) was determined by bone marrow biopsy. In addition to increased production of the myeloid and megakaryocytic lines, abnormal aggregation and grouping of megakaryocytes were seen. Reticular fibers were increased. He had some benefit of dyphenilhydantoin treatment given for the painful cramps in his neck and forearm muscles. Hydroxyurea treatment was started for myelofibrosis. Six months later, his general condition was better, and the painful cramps were completely resolved. No marked deterioration has been detected in neurologic examination and electromyography for 1 year.

Atrophy↗

Diagnostic value of CT in the detection of brain metastasis in small cell lung cancer patients.

Fifty-seven patients with histologically proven small cell lung carcinoma were prospectively evaluated for signs of brain metastasis by neurological examination and computerized tomographic (CT) brain scanning. The reliability of demonstrating brain metastases by means of neurological examination was compared with that of CT. Three (5%) patients, two with cerebrovascular infarcts and one with leptomeningeal metastases, were excluded from the study. Four (7%) patients, all with extensive disease, showed neurological symptoms and signs of brain metastases, which were confirmed in three cases by brain CT. The fourth patient had neurological symptoms and signs pointing to cerebral metastasis, but no sign of this was detected on CT at the time of diagnosis. However, six months later, after completion of chemotherapy, CT revealed signs of brain metastasis. The other fifty neurologically asymptomatic patients had no brain metastases on CT. This clinical study suggests that routine CT of the brain is not useful in neurologically asymptomatic patients with small cell lung carcinoma.

Aged↗

The Mt. Diwata study on the Philippines 1999--assessing mercury intoxication of the population by small scale gold mining.

The region of Diwalwal, dominated by Mt. Diwata, is a gold rush area on Mindanao (Philippines) where approximately 15000 people live. The fertile plain of Monkayo is situated downstream, where people grow crops such as rice and bananas; locally caught fish is eaten frequently. The ore is dug in small-scale mines and ground to a powder by ball-mills while still in Diwalwal. The gold is then extracted by adding liquid mercury (Hg), forming gold-amalgam. To separate the gold from the Hg, in most cases the amalgam is simply heated in the open by blow-torches. A high external Hg burden of the local population must be assumed. To evaluate the internal Hg burden of the population and the extent of possible negative health effects, 323 volunteers from Mt. Diwalwal, Monkayo and a control group from Davao were examined by a questionnaire, neurological examination and neuro-psychological testing. Blood, urine and hair samples were taken from each participant and analyzed for total Hg. A statistical evaluation was possible for 102 workers (occupationally Hg burdened ball-millers and amalgam-smelters), 63 other inhabitants from Mt. Diwata ('only' exposed from the environment), 100 persons, living downstream in Monkayo, and 42 inhabitants of Davao (serving as controls). The large volume of data was reduced to yes/no decisions. Alcohol as a possible bias factor was excluded (level of alcohol consumption and type, see Section 4.4). Each factor with a statistically significant difference of at least one exposed group to the control group was included in a medical score (0-21 points). In each of the exposed groups this score was significantly worse than in the control group (median control, 3; downstream, 9; Mt. Diwata, non-occupational exposed, 6; Hg workers, 10). In comparison to the surprisingly high Hg concentration in blood (median, 9.0 microg/l; max, 31.3) and in hair (2.65 microg/g; max, 34.7) of the control group, only the workers show elevated levels: Hg-blood median 11.4, max 107.6; Hg-hair median 3.62, max 37.8. The Hg urine concentrations of the occupational exposed and non-exposed population on Mt. Diwata was significantly higher than in the control group: control median 1.7 microg/l, max 7.6; non-occupational burdened median 4.1, max 76.4; and workers median 11.0, max 294.2. The participants, living downstream on the plain of Monkayo show no statistically significant difference in Hg-blood, Hg-urine or Hg-hair in comparison with the control group. The German Human-Biological-Monitoring value II (HBM II) was exceeded in 19.5% (control), 26.0% (downstream), 19.4% (Mt. Diwata, non-occupational) and 55.4% (workers) of the cases, the German occupational threshold limit in 19.6% of the workers. Only some of the clinical data, characteristic for Hg intoxication (e.g. tremor, loss of memory, bluish discoloration of the gingiva, etc.), correlate with Hg in blood or urine, but not with Hg in hair. The medical score sum correlates only with Hg in urine. The poor correlation between the Hg concentration in the biomonitors to classic clinical signs of chronic Hg intoxication may be explained by several factors: Hg in blood, urine and hair do not adequately monitor the Hg burden of the target tissues, especially the brain. Inter-individual differences in the sensitiveness to Hg are extremely large. In this area a mixed burden of Hg species must be assumed (Hg vapor, inorganic Hg, methyl-Hg). Chronic Hg burden may have established damage months or even years before the actual determination of the Hg concentrations in the bio-monitors under quite different burden was performed (Drasch G. Mercury. In: Seiler HG, Sigel A, Sigel H, editors. Handbook on metals in clinical and analytical chemistry. New York: Marcel Dekker, 1994:479-494). Therefore, a 'Hg intoxication', that should be treated, was not diagnosed by the Hg concentration in the bio-monitors alone, but by a balanced combination of these Hg values and the medical score sum. In principle, this means the higher the Hg concentration in the bio-monitors, the lower the number of characteristic adverse effects are required for a positive diagnosis. By this method, 0% of the controls, 38% downstream, 27% from Mt. Diwata, non-occupational exposed and 71.6% of the workers were classified as Hg intoxicated. A reduction of the external Hg burden on Mt. Diwata is urgently recommended. An attempt to treat the intoxicated participants with the chelating agent dimercaptopropanesulfonic acid (DMPS) is planned.

Adolescent↗

Neurological manifestations in Wilson's disease: Report of 119 cases.

We describe the neurological manifestations of 119 patients with WD (93 index cases and 26 affected family members) seen between 1963 and 2004. The mean age at symptoms onset was 19.6 years (range, 7-37 years). Medical records were reviewed for the patient's first neurological examination. The most frequent neurological manifestations observed were dysarthria (91%), gait disturbance (75%), risus sardonicus (72%), dystonia (69%), rigidity (66%), tremor (60%), and dysphagia (50%). Less frequent manifestations were chorea (16%) and athetosis (14%). Rare neurological presentations were seizures (4.2%), and pyramidal signs (3%).

Adolescent↗

Neurological status, sleep apnea frequency and blood oxygenation in six weeks old infants.

A study was carried out to investigate the correlation between sleep apnea frequency, blood oxygenation and neurological condition in 21 infants at six weeks of age with inconspicuous medical history. Polygraphic recordings of respiratory behaviour and transcutaneous blood gas monitoring lasted for at least five hours. To quantify the results of the neurological examination we established a neurological optimality score (NOS). We found statistically significant correlations between several indexes of apneas and indexes of blood oxygenation and NOS. Thus, a reduced NOS was strongly correlated with higher apnea frequencies and with pronounced drops of transcutaneous PO2-values. In addition all but one infant who were diagnosed as having a sleep apnea syndrome showed a greater than ten percent reduction of NOS. Some pathophysiological considerations to explain our findings are put forward and the possible role of the neuromodulator adenosine is emphasized.

Humans↗

Neurologic evaluation of the ear.

Diseases of the ear often cause signs of neurologic dysfunction because of damage of peripheral nervous system structures associated with the middle and inner ear. Vestibular dysfunction, facial paralysis, Horner's syndrome, and hearing deficits are the most common neurologic deficits that accompany middle and inner ear disease. Differentiating these signs from disease of the central nervous system is crucial for an accurate diagnosis and prognosis but can be difficult. Understanding the normal anatomy of the ear and its association with the brain is crucial to interpretation of the neurologic examination. This article reviews neurologic dysfunction commonly associated with diseases of the ear and differentiating these signs from central disease.

Animals↗

Neurologic condition of healthy term infants at 18 months: positive association with venous umbilical DHA status and negative association with umbilical trans-fatty acids.

Prenatal long-chain polyunsaturated fatty acids (LCPUFAs) and trans-fatty acids may affect neurodevelopment. In healthy term children, we determined relationships between relative fatty acid contents of umbilical arteries and veins and neurodevelopment at 18 mo. The study comprised a mixed group of 317 breast-fed, formula-fed, and LCPUFA formula-fed children. Study endpoints were the Hempel neurologic examination resulting in a neurologic classification and neurologic optimality score (NOS), and the Bayley Psychomotor Developmental Index (PDI) and Mental Developmental Index (MDI). Fifteen children showed minor neurologic dysfunction (MND). The umbilical vein trans, trans-18:2n-6 content was higher in children with MND than in the normal group. The NOS was significantly reduced in infants with an umbilical vein docosahexaenoic acid (DHA) content within the lowest quartile. Umbilical vein arachidonic acid (AA) was related to NOS in univariate statistics but not in multivariate analyses. The sum of trans-fatty acids and that of C18 trans-fatty acids showed a negative association with NOS in both univariate and multivariate analyses. No associations were found between AA, DHA and total trans-fatty acids with PDI or MDI. In conclusion, neonates with a relatively low DHA status and those with high trans-fatty acid levels have a less favorable neurologic condition at 18 mo.

Dietary Fats, Unsaturated↗

Problems in neurolocalization.

A complete neurologic examination is essential to manage patients with thoracolumbar or cervical intervertebral disc herniations appropriately. The neurologic examination provides an assessment of the severity of the lesion and allows the lesion to be accurately localized. This chapter will review the basic principles of localizing the compressive spinal and lesions and will discuss some specific problems encountered in localizing thoracolumbar and cervical intervertebral disc herniations.

Animals↗