Neurologic Examination

Long-term survival rates even after successful resuscitation from out-of-hospital cardiac arrest are dismal. Most of those initially resuscitated expired during their hospitalization. Recent reports have suggested that a more aggressive approach to postresuscitation care is the key to better outcome. Waiting for the evidence of neurological recovery before acting can result in missed opportunity to improve such recovery. Immediate induction of mild therapeutic hypothermia for all resuscitated victims who remain comatose offers the best hope for neurological recovery. Numerous reports suggest that early coronary angiography and PCI also improve outcome among those resuscitated from cardiac arrest whose postresuscitation ECG show evidence of ST elevation myocardial infarctions. Most promising is combining these two postresuscitation therapies, namely immediate induction of hypothermia and early coronary angiography and PCI. Combining these therapies has resulted in long-term survival rates of 70% with more than 80% of all such survivors neurologically functional. Even those without ST elevation on their postresuscitation ECG can greatly benefit from timely induction of hypothermia and early angiography/PCI. V C 2009 Wiley-Liss, Inc.

Object. Surgery in children with epilepsy is a new, evolving field. The important practical issues have been to define strategies for choosing the most suitable candidates and the type and optimal timing of epilepsy surgery. This study was undertaken to elucidate these points.

Objective: In view of paucity of evoked potential changes in Pott's paraplegia, it is proposed to evaluate the role of motor and somatosensory evoked potentials in predicting the outcome. Methods: Consecutive patients with Pott's paraplegia during 1993-2003 were subjected to detailed clinical, radiological and evoked potential study. The latter comprised of tibial somatosensory evoked potential (SEP) and motor evoked potential (MEP) study to tibialis anterior. The patients were clinically evaluated at 6 and 12 months and the outcome was defined into poor (bed ridden), partial (dependent for activities of daily living) and complete recovery (independent). The evoked potential findings were correlated with clinical and radiological findings and outcome. Results: There were 39 patients whose age ranged between 16 and 70 (mean 42.1) years and 22 were females. The mean duration of symptoms was 8.2 months. Sensory motor deficit was present in 18 and pure motor signs in 21 patients. Five patients had quadriplegia and remaining had paraplegia. The muscle weakness was severe in 12 and moderate in 15 patients. In 12 patients, lower limb power was normal but they had lower limb hyper-reflexia with or without spasticity suggesting pyramidal dysfunction. Pinprick and joint position sensations were abnormal in 18 patients. MRI was abnormal in all and revealed cervical involvement in 7, thoracic in 22 and lumbar in 10 patients. Paravertebral soft tissue shadow was present in 36 and cord compression in 30 patients. Motor evoked potential was abnormal in 19 patients (unrecordable in 11 patients, 21 sides and prolonged in 8 patients, 14 sides). SEP was abnormal in 18 patients (unrecordable in 15 patients, 25 sides and prolonged central conduction in 8 patients, 9 sides). Both MEP and SEP were abnormal in 16, normal in 18, and only MEP was abnormal in 3 and only SEP in 2 patients. At 6 month 25 patients had complete, 9 partial and 5 poor recovery. At 1 year 33 had complete and 4 partial recovery. SEP and MEP abnormalities correlated with respective sensory and motor functions, vertebral level and outcome at 6 and 12 months. Conclusions: MEP and SEP both are helpful in predicting 6-month outcome. Combining SEP and MEP gives stronger correlation with 6-month outcome compared to only MEP or SEP. The potential role of evoked potentials in deciding different therapeutic strategies needs further studies.

The user has requested enhancement of the downloaded file. 52 53 54 Commentary 55 This report reminds me of the time I spent at Tripler 56 Army Medical center in Honolulu during the mid-1960s. It 57 was there I initially encountered moxibustion and cupping. 58 Both techniques continue to be used to the present time by 59 traditional oriental medical practitioners [1-4]. Moxibustion 60 entails lighting a fire to a compressed, dried wad or stick 61 of mugwort, called a moxa, and holding it close to the skin 62 at accepted acupuncture sites or near the area of pain. The 63 heat causes increased capillary blood flow in the skin, thus 64 changing the energy flow and counteracting the pain or 65 disease. Cupping attempts the same thing by applying the 66 inverted cup to the skin and producing a vacuum, raising a 67 weal and increasing the capillary flow. Currently, cups are 68 usually made of glass and the vacuum applied through a 69 rubber bulb or mechanical suction attached to the inverted 70 bottom of the cup and the weal is temporary without cups were made of metal and the inverted bottom was a 73 hollow into which the moxa was placed and lit with a 74 match. The heat expanded the air in the cup, driving it out 75 where the cup was applied to the skin and suction 76 developed from the resulting vacuum also causing a weal. 77 However, the heat transmitted to the skin by the metal cup 78 often left permanent burn scars quite similar in shape and size 79 as those depicted in this letter to the editor. When I left the 80 military and started practice on the northeast side of Chicago, 81 the hospital I worked at was adjacent to an oriental 82 community. I consulted on a case of lumbar radiculopathy 83 in an elderly lady of Chinese descent who had been treated by 84 a traditional practitioner using moxibustion cupping. She had 85 a series of darkened circular scars, each about an inch wide 86 running linearly down the back of her thigh and calf to the 87 ankle, following her radicular pain, as is noted in this report 88 by Rahimi-Movaghar. 89 The Balouch people of Iran have a long history, extending 90 back to either the original people of the fertile crescent or to 91 immigrants from the area around the Caspian Sea [5]. They 92 are not directly related to the ancient Chinese and it appears 93 that the use of heat to treat maladies arose independent of 94 Chinese tradition and thus is likely an even more ancient 95 human tradition. I agree with the author's goal of trying to 96 educate people to more acceptable modern ways of medical 97

A 68-year-old woman and a 72-year-old man presented with distal weakness of the limbs and numbness following an influenza vaccination within 2 weeks. Moreover, Guillain-Barré syndrome (GBS) was diagnosed in two patients. Pericarditis was diagnosed in the first patient who also had precordial chest pain with referral to trapezius ridge, and nephrotic syndrome, was observed in the second patient who had leg edema and proteinuria. The relationship among GBS, pericarditis and nephrotic syndrome after an influenza vaccination is discussed.

There is limited information about the effect of erythropoietin or a high hemoglobin transfusion threshold after a traumatic brain injury. OBJECTIVE To compare the effects of erythropoietin and 2 hemoglobin transfusion thresholds (7 and 10 g/dL) on neurological recovery after traumatic brain injury. DESIGN, SETTING, AND PARTICIPANTS Randomized clinical trial of 200 patients (erythropoietin, n = 102; placebo, n = 98) with closed head injury who were unable to follow commands and were enrolled within 6 hours of injury at neurosurgical intensive care units in 2 US level I trauma centers between May 2006 and August 2012. The study used a factorial design to test whether erythropoietin would fail to improve favorable outcomes by 20% and whether a hemoglobin transfusion threshold of greater than 10 g/dL would increase favorable outcomes without increasing complications. Erythropoietin or placebo was initially dosed daily for 3 days and then weekly for 2 more weeks (n = 74) and then the 24-and 48-hour doses were stopped for the remainder of the patients (n = 126). There were 99 patients assigned to a hemoglobin transfusion threshold of 7 g/dL and 101 patients assigned to 10 g/dL. INTERVENTIONS Intravenous erythropoietin (500 IU/kg per dose) or saline. Transfusion threshold maintained with packed red blood cells. MAIN OUTCOMES AND MEASURES Glasgow Outcome Scale score dichotomized as favorable (good recovery and moderate disability) or unfavorable (severe disability, vegetative, or dead) at 6 months postinjury. RESULTS There was no interaction between erythropoietin and hemoglobin transfusion threshold. Compared with placebo (favorable outcome rate: 34/89 [38.2%; 95% CI, 28.1% to 49.1%]), both erythropoietin groups were futile (first dosing regimen: 17/35 [48.6%; 95% CI, 31.4% to 66.0%], P = .13; second dosing regimen: 17/57 [29.8%; 95% CI, 18.4% to 43.4%], P < .001). Favorable outcome rates were 37/87 (42.5%) for the hemoglobin transfusion threshold of 7 g/dL and 31/94 (33.0%) for 10 g/dL (95% CI for the difference, −0.06 to 0.25, P = .28). There was a higher incidence of thromboembolic events for the transfusion threshold of 10 g/dL (22/101 [21.8%] vs 8/99 [8.1%] for the threshold of 7 g/dL, odds ratio, 0.32 [95% CI, 0.12 to 0.79], P = .009). CONCLUSIONS AND RELEVANCE In patients with closed head injury, neither the administration of erythropoietin nor maintaining hemoglobin concentration of greater than 10 g/dL resulted in improved neurological outcome at 6 months. The transfusion threshold of 10 g/dL was associated with a higher incidence of adverse events. These findings do not support either approach in this setting.

A cranioplasty has long been thought to be therapeutic. This patient confirms this by illustrating a serious complication that may occur following cerebellar herniation through an occipital defect. It should alert readers to the fact that although there may be no immediate complications associated with not closing the dura or replacing the bone flap, the uncommon problem described in this paper is serious and potentially life-threatening. Thus, we recommend adequate dural and bony closure for all intracranial surgery, particularly in the posterior fossa. We are not aware of a similar report following posterior fossa surgery.

To develop and validate a patientbased instrument to measure both facial impairment and disability, the Facial Clinimetric Evaluation (FaCE) Scale. Study Design: Prospective instrument validation. Methods: Eighty-six patients with a documented history of facial paralysis completed a preliminary, 51-item instrument (alpha FaCE Scale), as well as the previously developed Facial Disability Index (FDI) and the Medical Outcomes Study Short Form 36 Item Questionnaire (SF-36). Two weeks after completing these instruments, 76 patients again completed the alpha FaCE Scale. Forty-one of the patients were also evaluated using the House-Brackmann Grading System (HBGS) and the Facial Grading System (FGS). Results: Exploratory principal component factor analysis grouped 15 FaCE Scale items into 6 impairment and disability categories (domains), forming the beta FaCE Scale. Overall, the test-retest reliability of the FaCE Scale was high (Spearman's correlation coefficient (r) ‫؍‬ 0.88, P <.01), as were the reliability coefficients of the individual domains (r ‫؍‬ 0.81-0.92, P <.01). The FaCE Scale domains showed appropriate correlation to global visual analogue scale questions posed on the original alpha FaCE Scale (r ‫؍‬ 0.65-0.81, P <.01). Overall, the FaCE Scale showed significant correlation with HBGS and FGS scores (r ‫؍‬ ؊0.55 and 0.57, respectively; P <.01). However, not all FaCE Scale domains correlated with the HBGS and FGS scores. Conclusions: A reliable and valid patientbased system to measure impairment and disability in facial paralysis has been developed. This system appears to be better than traditional, physiciangraded scales for evaluating quality-of-life issues affected by facial disability.

Determination of a scale's factor structure requires a two-part process: (1) an initial examination of the factor structure using a sample of individuals with the condition of interest, and (2) repeated examinations of the factor structure using the same analytic methods but applied to independent samples of individuals with the condition of interest who contribute unique variability to the scale measurement. In a previous study, we performed an initial investigation of the factor structure of the Motor Examination section of the Unified Parkinson's Disease Rating Scale (UPDRS). We used a sample of 294 consecutive patients with idiopathic Parkinson's disease (PD) assessed while in the on-state and identified six clinically distinct factors. In the present study, we performed a confirmatory investigation of the factor structure and analysis of the internal consistency of the UPDRS in a new sample of 200 consecutive PD patients who were assessed while in the offstate. Factor analysis again revealed six factors with identical item loadings as those obtained from examinations of patients in the on-state. Estimates of internal consistency were comparable in the off-and on-state examinations. These results indicate that the Motor Examination section of the UPDRS has a stable factor structure and high internal consistency across offand on-state examinations.

Objectives: We investigated intra-and inter-evaluator reliability to quantify spasticity based on the tonic stretch reflex threshold (TSRT) and the correlation between TSRT and resistance to stretch. Methods: Spasticity was evaluated in 20 subjects with chronic stroke-related spasticity using a portable device and the Modified Ashworth Scale (MAS). Evaluations were done on 2 days, by three evaluators. Biceps brachii EMG signals and elbow displacement were recorded during 20 elbow stretches applied at different velocities for each evaluation. Velocity-dependent dynamic stretch reflex thresholds (angle where EMG signal increased in the biceps for a given velocity of stretch) were recorded. These values were used to compute TSRT (excitability of motoneurons at 0°/s). Spasticity was also measured with MAS. Results: Reliability was moderately good for subjects with moderate to high spasticity (intra-evaluator: 0.46-0.68, and inter-evaluator: 0.53-0.68). The TSRT measure of spasticity did not correlate with resistance to stretch (MAS). Conclusions: TSRT may be a more representative measure for subjects with moderate to high spasticity. Further improvements are suggested for the portable device in order to quantify all the levels of spasticity. Significance: TSRT may be an alternative clinical measure to current clinical scales.

Postural adjustments associated with the task of rising on tiptoes were investigated in a reaction time paradigm in 10 normal subjects and 18 patients with cerebellar disorders. Cerebellar dysfunction was due to either degenerative cerebellar disease, tumor, or ischemia. Displacements of the center of foot pressure (CFP) were recorded. The task, accomplished by the triceps surae muscle (executional activity, mean latency of 41 1 ms), is mechan

Rabin A, Gerszten PC, Karausky P, Bunker CH, Potter DM, Welch WC. The sensitivity of the seated straight-leg raise test compared with the supine straight-leg raise test in patients presenting with magnetic resonance imaging evidence of lumbar nerve root compression. Arch Phys Med Rehabil 2007;88:840-3.

O ut-of-hospital cardiac arrest (OHCA) is still associated with a poor prognosis despite improvement in survival in recent years. 1,2 In a previous study, we found a higher survival rate after OHCA and admission to tertiary heart centers compared with admission to nontertiary hospitals. 3 The difference in survival has also been found in several other studies from different countries, which has not been explained by differences in prehospital circumstances only. In the recent advanced life support guidelines, postresuscitation care has been included in the chain-of-survival emphasizing the treatment and prevention of the metabolic and hemodynamic disturbances that often develop as part of postcardiac arrest syndrome. The reasons for the difference in survival in tertiary centers versus nontertiary hospitals are largely unknown and have only been speculative. The establishment of high-volume cardiac arrest centers with implementation of postresuscitation management protocols with targeted temperature management, Background-Studies have found higher survival rates after out-of-hospital cardiac arrest and admission to tertiary heart centers. The aim was to examine the level-of-care at tertiary centers compared with nontertiary hospitals and the association with outcome after out-of-hospital cardiac arrest. Methods and Results-Consecutive out-of-hospital cardiac arrest patients (n=1078) without ST-segment-elevation myocardial infarction admitted to tertiary centers (54%) and nontertiary hospitals (46%) were included (2002)(2003)(2004)(2005)(2006)(2007)(2008)(2009)(2010)(2011)). Patient charts were reviewed focusing on level-of-care and comorbidity. Survival to discharge differed significantly with 45% versus 24% of patients discharged alive (P<0.001), and after adjustment for prognostic factors admissions to tertiary centers were still associated with lower 30-day mortality (hazard ratio, 0.78 [0.64-0.96; P=0.02]), independent of comorbidity. The adjusted odds of predefined markers of level-of-care were higher in tertiary centers: admission to intensive care unit (odds ratio [OR], 1.8 [95% confidence interval, 1.2-2.5]), temporary pacemaker (OR, 6.4 [2.2-19])

Pyogenic spondylodiscitis associated with epidural abscess is a rare but serious problem in spinal surgery, because it may cause a severe morbidity or mortality, if the diagnosis is established late and the treatment is inadequate. A case of pyogenic thoracic spondylodiscitis associated with epidural abscess whose symptoms progressed over two months from back pain to acute paraplegia was presented. Magnetic resonance imaging of the spine suggested the presence of T9–10 spondylodiscitis with partial destruction of the T9 and T10 vertebral bodies and concomitant epidural abscess. Treatment consisting of surgical debridement of infected vertebrae and disc material, fusion and anterior spinal instrumentation was performed. Microbiological culture of the material revealed infection with Staphylococcus aureus and after 3 months of antibiotic treatment, recovery was almost complete. Based on a thorough review of the literature and the case presented in this report, it is concluded that accurate and prompt diagnosis requires high index of suspicion followed by a combination of adequate surgical and conservative treatment prevents severe morbidity in cases of nonspecific pyogenic spondylodiscitis associated with epidural abscess.

A chronic, and at times, progressive neurologic syndrome associated with boxing has been recognized for some time by boxing fans and people involved with the sport. Since Martland&amp;#39;s first description of the syndrome in 1929, there has been only one randomly selected study of ex-boxers, that of Roberts, which showed a 17 per cent prevalence of this syndrome among boxers who fought between 1929 and 1955. This syndrome can be progressive but often is not. Furthermore, the extent of occupational exposure is probably a significant risk factor. Because of this, it would be expected that the prevalance of the syndrome in the modern boxer, as well as the amateur, would be significantly less than during the first half of the century, and, indeed, several studies appear to support this. Recent studies provide evidence that brain damage does exist in modern boxers and suggests that &amp;quot;subclinical&amp;quot; brain damage is likely to be more prevalent than obvious clinical dysfunction. There is clearly a discrepancy between subclinical evidence of neurologic involvement (for example, an abnormal CT scan) and signs of clinical neurologic dysfunction (for example, clinical exam and neuropsychological testing). The latter tend to show less frequent and consistent evidence of brain damage in boxers than does the CT scan. Although it is tempting to assume that an abnormal CT scan presages the development of neurologic dysfunction, it is not clear that this is the case. The prevalence of the syndrome, risk for progression to functional deficit, warning signs, and the natural history cannot be defined at this time. The only way to better define these parameters would be a controlled prospective study, which has yet to be undertaken.

We questioned whether neuron-specific enolase (NSE) and myelin basic protein (MBP) concentrations in cerebrospinal fluid (CSF) in the first 72 hours of life are correlated with the neurologic condition of asphyxiated full-term infants in the neonatal period and at 1 year of age. Sixty-nine asphyxiated infants were studied with serial neurologic examination, cranial ultrasonography, and neurologic follow-up. CSF samples were obtained by lumbar puncture at 12 and 72 hours of life. NSE was measured by enzyme immunoassay, and MBP was measured by radioimmunoassay. Twenty infants had no neonatal encephalopathy and 49 exhibited different stages of encephalopathy. NSE and MBP concentrations in CSF at 12 and 72 hours of life were related to the degree of neonatal encephalopathy. Neither NSE nor MBP levels were correlated with any perinatal factors. Infants with documented brain injury had the highest concentrations of both NSE and MBP. The concentrations of these two biochemical markers at b...

Background Traumatic spinal cord injury is a serious disorder in which early prediction of ambulation is important to counsel patients and to plan rehabilitation. We developed a reliable, validated prediction rule to assess a patient's chances of walking independently after such injury.

In recent studies aimed at assessing the effects of original therapeutic strategies applied to patients with Huntington's disease (HD), we observed informative changes in electrophysiological results that recovered normal values in coherence with clinical improvement. However, longitudinal studies were lacking for determining whether electrophysiological test results evolve in parallel with clinical markers of the natural course of the disease and could consequently provide objective quantifiable markers of disease progression. For this purpose, electrophysiological testing was performed annually in a cohort of 20 patients with HD over a 2-year period (three examinations). The study included the recording of sympathetic skin responses and blink reflexes (BRs) to supraorbital nerve stimulation, long latency reflexes (LLRs) and somatosensory evoked potentials (SEPs) to median nerve stimulation, and cortical silent periods (CSPs) to transcranial magnetic stimulation. Clinical evaluation was based on the Total Functional Capacity scale (TFC) and the Motor part of the Unified Huntington's Disease Rating Scale (UHDRS). A significant deterioration with time was found for BR latency, LLR presence, various SEP parameters (parietal N20 peak amplitude and frontal N30 presence) and CSP duration. Attenuation of the N20 peak and CSP shortening correlated with functional decline, as assessed by the TFC score, whereas delayed BR and LLR abolition correlated with UHDRS Motor score deterioration. This study shows that several electrophysiological parameters are closely associated with dysfunction of various neural circuits in HD and could be useful markers of disease progression.

Children with Crigler-Najjar syndrome type I are at increased risk for neurologic deficits. Cerebellar symptoms are not prominent and appear in adolescent or adult patients with this syndrome. We report a 2-yearold female with Crigler-Najjar syndrome type I who presented severe cerebellar symptoms revealing bilirubin encephalopathy. The patient improved slowly with the duration of phototherapy. Cerebellar symptoms can be the initial manifestation of kernicterus in children with Crigler-Najjar syndrome type I.

Pha1b is a potent toxin obtained from the spider Phoneutria nigriventer that blocks neuronal voltage-sensitive Ca 2þ channels. This study compared the antiallodynic effects of Pha1b, u-conotoxin MVIIA and morphine in mice and their side effects in rats. Mechanical allodynia was measured in mice receiving single intrathecal administration of Pha1b, u-conotoxin MVIIA or morphine before or after the incisional plantar procedure. The effect of the treatments on cardiovascular profile and global neurological were evaluated in rats. The expression of pro or anti-inflammatory cytokines of human polymorph mononuclear cells was also evaluated.

Approximately 50% of human spinal cord injuries (SCI) are at the cervical level, resulting in impairments in motor function of the upper extremity. Even modest recovery of upper extremity function could have an enormous impact on quality of life for quadriplegics. Thus, there is a critical need to develop experimental models for cervical SCI and techniques to assess deficits and recovery of forelimb motor function. Here, we analyze forelimb and forepaw motor function in rats after a lateral hemisection at C5 and assessed the relationship between the functional impairments and the extent of damage to one descending motor system, the corticospinal tract (CST). Female Sprague-Dawley rats were trained on various behavioral tasks that require the forelimb, including a task that measures gripping ability by the hand (as measured by a grip strength meter, GSM), a food reaching task, and horizontal rope walking. After 8 weeks of post-injury testing, the distribution of the CST was evaluated by injecting BDA into the sensorimotor cortex either ipsi-or contralateral to the cervical lesion. Complete unilateral hemisection injuries eliminated the ability to grip and caused severe impairments in food retrieval by the forepaw ipsilateral to the lesion. There was no indication of recovery in either task. In cases in which hemisections spared white matter near the midline, there was some recovery of forelimb motor function over time. Assessment of rope climbing ability revealed permanent impairments in forelimb use and deficits in hindlimb use and trunk stability. Sensory testing using a dynamic plantar aesthesiometer revealed that there was no increase in touch sensitivity in the affected forelimb. For the cases in which both histological and behavioral data were available, spared forelimb motor function was greatest in rats in which there was sparing of the dorsal CST. D

The goals of an international taskforce on somatosensory testing established by the Special Interest Group of Oro-facial Pain (SIG-OFP) under the International Association for the Study of Pain (IASP) were to (i) review the literature concerning assessment of somatosensory function in the oro-facial region in terms of techniques and test performance, (ii) provide guidelines for comprehensive and screening examination procedures, and (iii) give recommendations for future development of somatosensory testing specifically in the oro-facial region. Numerous qualitative and quantitative psychophysical techniques have been proposed and used in the description of oro-facial somatosensory function. The selection of technique includes time considerations because the most reliable and accurate methods require multiple repetitions of stimuli. Multiple-stimulus modalities (mechanical, thermal, electrical, chemical) have been applied to study oro-facial somatosensory function. A battery of different test stimuli is needed to obtain comprehensive information about the functional integrity of the various types of afferent nerve fibres. Based on the available literature, the German Neuropathic Pain Network test battery appears suitable for the study of somatosensory function within the oro-facial area as it is based on a wide variety of both qualitative and quantitative assessments of all cutaneous somatosensory modalities. Furthermore, these protocols have been thoroughly described and tested on multiple sites including the facial skin and intra-oral mucosa. Standardisation of both comprehensive and screening examination techniques is likely to improve the diagnostic accuracy and facilitate the understanding of neural mechanisms and somatosensory changes in different oro-facial pain conditions and may help to guide management.

Background. This prospective study, conducted in patients with Chiari I malformation (C I) related syringomyelia who underwent posterior decompression and duroplasty, utilizes radionuclide cisternography in order to study the cerebrospinal fluid (CSF) dynamics at the foramen magnum and to predict the clinical outcome following surgery. Methods. 17 consecutive patients of C I with syringomyelia (but without hydrocephalus or fixed atlanto-axial dislocation), underwent a detailed neurological examination and were assigned a clinical disability score based on the modified Klekamp and Samii score. A radionuclide cisternography (using Tc99m-DTPA) was performed via the lumbar route and the ascent of the tracer was followed utilizing a gamma camera immediately after injection and then sequentially after 1, 2, 4, 6 and 24 hours. After posterior decompression and duroplasty, the modified Klekamp and Samii score was repeated at follow-up visits (range: 3 months to one year) along with radionuclide cisternography at 3 months, and MR imaging at 6 months. Findings. Three patterns of tracer flow were observed: a) rapid flow (n=7); b) supratentorial subarachnoid delay (n=7); and, c) foramen magnum block (n=3). The patients having foramen magnum block had the poorest clinical scores on admission. At follow up, there was an improvement in the clinical scores so that the mean scores in all three categories reached nearly the same level. Following posterior decompression, the radionuclide cisternography performed in 10 patients showed a rapid flow of the tracer without any obstruction. The syrinx resolved in 4 of the 11 patients in whom an MRI was done. Interpretation. The patients with C I with syringomyelia may often have a free flow of tracer across the FM. Posterior decompression and duroplasty provides maximum clinical relief in patients with a demonstrable foramen magnum block on radionuclide cisternography while those with a normal flow have less relief. The symptomatology related to brain-stem compression immediately responds to the surgical procedure but the syrinx-induced signs and symptoms of spinal cord dysfunction persist.

Hammersmith Infant Neurological Examination (HINE) is a set of tests used for grading neurological development of infants on a scale of 0 to 3. These tests help in assessing neurophysiological development of babies, especially preterm infants who are born before (the fetus reaches) the gestational age of 36 weeks. Such tests are often conducted in the follow-up clinics of hospitals for grading infants with suspected disabilities. Assessment based on HINE depends on the expertise of the physicians involved in conducting the examinations. It has been noted that some of these tests, especially pulled-to-sit and lateral tilting, are difficult to assess solely based on visual observation. For example, during the pulled-to-sit examination, the examiner needs to observe the relative movement of the head with respect to torso while pulling the infant by holding wrists. The examiner may find it difficult to follow the head movement from the coronal view. Video object tracking based automatic or semi-automatic analysis can be helpful in this case. In this paper, we present a video based method to automate the analysis of pulled-to-sit examination. In this context, a dynamic programming and node pruning based efficient video object tracking algorithm has been proposed. Pulled-to-sit event detection is handled by the proposed tracking algorithm that uses a 2-D geometric model of the scene. The algorithm has been tested with normal as well as marker based videos of the examination recorded at the neuro-development clinic of the SSKM Hospital, Kolkata, India. It is found that the proposed algorithm is capable of estimating the pulled-to-sit score with sensitivity (80%-92%) and specificity (89%-96%).

Retrospective review of prospective database. To define the variability of neurologic examination and recovery after nonpenetrating complete thoracic spinal cord injuries (American Spinal Injury Association [ASIA] A). Neurologic examinations after spinal cord injury (SCI) can be difficult and inconsistent. Unlike cervical SCI patients, alterations in thoracic (below T1) complete SCI (ASIA A-based on the ASIA Impairment Scale [AIS]) patients' examinations are based only on sensory testing, thus changes in the neurologic level (NL) are determined only by sensory changes. A retrospective review of the placebo control patients in a multicenter prospective database used for the pharmacologic trial of Sygen. Patients were included if they had a complete thoracic SCI on initial evaluation, with completed ASIA examinations at follow-up weeks 4, 8, 16, 26, and 52. Specifically, pin prick (PP) and light touch (LT) were assessed and the absolute change was calculated as the number of spinal levels at a given observation time. Three thousand one hundred sixty-five patients were initially screened for the Sygen clinical trial, of which 51 were the control placebo patients used in this analysis. Alterations from the baseline examination (PP and LT) were fairly consistent and the median change/recovery in neurologic examination was 1 spinal level. Across all observations postbaseline, the average change for PP was 1.48 +/- 0.13 (mean +/- SE), and for LT, 1.40 +/- 0.13. There were equal proportions of directional changes (none, improved, lost). Changes in a thoracic complete (ASIA A) SCI patients ASIA examination as measured through sensory methods (PP/LT) are fairly uncommon. The overall examination had only 1- to 2-level variability across patients, indicating minimal change in the sensory examination over the follow-up period. Stability in the ASIA examination as measured through sensory methods has thus been demonstrated over time, making it an excellent tool to monitor changes in neurologic function.

A female neonate with pyruvate dehydrogenase (PDH) deficiency is presented with clinical, radiologic, biochemical, neuropathoiogic, and molecular genetic data. She was dysmorphic, with a high forehead, lowset ears, thin upper lip, upturned nose, and rhizomelic limbs. Cranial MRI revealed severe cortical atrophy, ventricular dilatation, and corpus caliosum agenesis. Pyruvate and lactate levels were increased in CSF and blood. Urinary organic acid profile was compatible with PDH deficiency. PDH activity was normal in fibroblasts, lymphocytes, and muscle. The PDH El-or gene was sequenced and a single base mutation was found within the regulatory phosphorylation site in exon 10. It is postulated that this mutation causes a cerebral form of PDH deficiency. Tissue-specific expression of the disease could be explained by differential X chromosome inactivation because the PDH El-ct gene is located on this chromosome. Dysmorphism with severe cerebral malformations in female patients merits a metabolic evaluation, including determination of lactate and pyruvate levels in CSF.

Nerve injury is one of the most serious complications associated with limb-lengthening. We examined the risk, assessment, and treatment of nerve lesions associated with limb-lengthening. We retrospectively studied the records on 814 limb-lengthening procedures. Nerve lesions were defined by clinical signs and symptoms of motor function impairment, sensory alterations, referred pain in the distribution of an affected nerve, and/or positive results of quantitative sensory testing with use of a pressure specified sensory device. Seventy-six (9.3%) of the limbs had a nerve lesion. Eighty-four percent of the nerve lesions occurred during gradual distraction, and 16% occurred immediately following surgery. The pressure specified sensory device showed 100% sensitivity and 86% specificity in the detection of nerve injuries. The patients in whom the lesion was diagnosed with this method, or with this method as well as with nerve conduction studies, had significantly faster recovery than did ...

Background: Only few standardized apraxia scales are available and they do not cover all domains and semantic features of gesture production. Therefore, the objective of the present study was to evaluate the reliability and validity of a newly developed test of upper limb apraxia (TULIA), which is comprehensive and still short to administer. Methods: The TULIA consists of 48 items including imitation and pantomime domain of non-symbolic (meaningless), intransitive (communicative) and transitive (tool related) gestures corresponding to 6 subtests. A 6-point scoring method (0-5) was used (score range 0-240). Performance was assessed by blinded raters based on videos in 133 stroke patients, 84 with left hemisphere damage (LHD) and 49 with right hemisphere damage (RHD), as well as 50 healthy subjects (HS).

We reviewed detailed clinical features of 266 patients with idiopathic cervical dystonia, commonly called spasmodic torticollis. Mean age at onset (41 years), female-to-male ratio (1.9:1), clustering of onset between ages 30 and 59 (70%), familial history of dystonia (12%), and remissions (9.8%) were similar to those found in previous studies. In contrast to the single prior large clinical study of this disorder, no predominance of right-handers or significant thyroid disease was found. Pain, which occurred in 75% of patients and contributed to disability score (p <0.01), distinguishes this syndrome from all other focal dystonias. Pain was also strongly associated with constant (vs. intermittent) head turning, severity of head turning, and presence of spasm. Eighty-three percent of patients had deviation of the head of >75% of the time when sitting with the head unsupported (constant head deviation at rest). Of the 97% who had head turning, 81% also had head tilting in various combinations. The 23% with hand tremor had an older age at onset (mean, 46 vs. 41 years; p <0.05). An earlier age at onset (p <0.05) was seen in patients with a family history of dystonia (mean, 36 years), with trauma shortly preceding symptoms (mean, 36 years), with a change in the direction of head turning (mean, 30 years), and with remissions (mean, 33 years). Jerky movements or forced transient spasms of the head occurred in 62% of the patients, and these patients would be the ones for whom the designation “spasmodic torticollis” could logically apply. Therefore, because the cervical dystonia in 38% of patients is not spasmodic, we propose that the term “spasmodic torticollis” is not a completely appropriate designation for this condition. We suggest labeling the condition either idiopathic cervical dystonia or idiopathic dystonic torticollis, preferably the former for reasons explained in the text.