Functional assessment of stretch hyperreflexia in children with cerebral palsy using treadmill perturbations.

BACKGROUND: As hyperactive muscle stretch reflexes hinder movement in patients with central nervous system disorders, they are a common target of treatment. To improve treatment evaluation, hyperactive reflexes should be assessed during activities as walking rather than passively. This study systematically explores the feasibility, reliability and validity of sudden treadmill perturbations to evoke and quantify calf muscle stretch reflexes during walking in children with neurological disorders. METHODS: We performed an observational cross-sectional study including 24 children with cerebral palsy (CP; 6-16 years) and 14 typically developing children (TD; 6-15 years). Short belt accelerations were applied at three different intensities while children walked at comfortable speed. Lower leg kinematics, musculo-tendon lengthening and velocity, muscle activity and spatiotemporal parameters were measured to analyze perturbation responses. RESULTS: We first demonstrated protocol feasibility: the protocol was completed by all but three children who ceased participation due to fatigue. All remaining children were able to maintain their gait pattern during perturbation trials without anticipatory adaptations in ankle kinematics, spatiotemporal parameters and muscle activity. Second, we showed the protocol's reliability: there was no systematic change in muscle response over time (P = 0.21-0.54) and a bootstrapping procedure indicated sufficient number of perturbations, as the last perturbation repetition only reduced variability by ~ 2%. Third, we evaluated construct validity by showing that responses comply with neurophysiological criteria for stretch reflexes: perturbations superimposed calf muscle lengthening (P < 0.001 for both CP and TD) in all but one participant. This elicited increased calf muscle activity (359 ± 190% for CP and 231 ± 68% for TD, both P < 0.001) in the gastrocnemius medialis muscle, which increased with perturbation intensity (P < 0.001), according to the velocity-dependent nature of stretch reflexes. Finally, construct validity was shown from a clinical perspective: stretch reflexes were 1.7 times higher for CP than TD for the gastrocnemius medialis muscle (P = 0.017). CONCLUSIONS: The feasibility and reliability of the protocol, as well as the construct validity-shown by the exaggerated velocity-dependent nature of the measured responses-strongly support the use of treadmill perturbations to quantify stretch hyperreflexia during gait. We therefore provided a framework which can be used to inform clinical decision making and treatment evaluation.

Assessment of sacral spinal excitability using stimulus-response curves of the bulbocavernosus reflex.

OBJECTIVE: To analyze and quantify sacral spinal excitability through bulbocavernosus reflex (BCR) stimulus-response curves. METHODS: Thirty subjects with upper motor neuron lesions (UMN) and nine controls were included in this prospective, monocentric study. Sacral spinal excitability was assessed using stimulus-response curves of the BCR, modeled at different bladder filling volumes relative to the desire to void (as defined by the International Continence Society) during a cystometry. Variations in α (i.e. the slope of the stimulus-response curve) were considered as an indicator of the modulation of sacral spinal excitability. RESULTS: In all subjects, α increased during bladder filling suggesting the modulation of spinal sacral excitability during the filling phase. This increase was over 30% in 96.7% of neurological subjects and 88.9% of controls. The increase was higher before the first sensation to void in the neurological population (163.15%), compared to controls, (29.91%), p < 0.001. CONCLUSIONS: We showed the possibility of using BCR stimulus-response curves to characterize sacral spinal response with an amplification of this response during bladder filling as well as a difference in this response amplification in patients with UMN in comparison with a control group. SIGNIFICANCE: BCR, through stimulus-response curves, might be an indicator of pelvic-perineal exaggerated reflex response and possibly a tool for evaluating treatment effectiveness.

Evaluation of persons with suspected lumbosacral and cervical radiculopathy: Electrodiagnostic assessment and implications for treatment and outcomes (Part I).

Persons with back, neck, and limb symptoms constitute a major referral population to specialists in electrodiagnostic (EDX) medicine. The evaluation of these patients involves consideration of both the common and less common disorders. The EDX examination with needle electromyography (EMG) is the most important means of testing for radiculopathy. This test has modest sensitivity but high specificity and well complements imaging of the spine. Needle EMG in combination with nerve conduction testing is valuable in excluding entrapment neuropathies and polyneuropathy-conditions that frequently mimic radicular symptoms. In this first of a two-part review, the optimal EDX evaluation of persons with suspected radiculopathy is presented. In part two, the implications of EDX findings for diagnosis and clinical management of persons with radiculopathy are reviewed.

A 30 s test for quantitative assessment of a relative afferent pupillary defect (RAPD): the infrared pupillary asymmetry (IPA).

BACKGROUND: Detection of a relative afferent pupillary defect (RAPD) by the swinging-light test can be challenging in clinical practice (dark eyes, anisocoria, dark environment). We developed a new method of RAPD quantification based on the recording of the infrared pupillary asymmetry (IPA) with a standard optical coherence tomography (OCT) device. METHODS: The diagnostic value of the IPA for detection of the RAPD was determined by receiver-operating characteristic (ROC) curves and area under the curve (AUC). RESULTS: Twenty-nine subjects were included in this study (17 controls and 12 unilateral optic neuropathies). The IPA was significantly greater in unilateral optic neuropathies (0.39) compared to controls (0.18, p = 0.001). The diagnostic value was good with a ROC-AUC of 0.843. Importantly, the IPA correlated significantly with the inter-eye percentage difference of the macular ganglion cell-inner plexiform layer (mGCIPL) thickness (R = 0.53, p = 0.01). Assessment of the IPA took less than 30 s. CONCLUSION: The present data show that the IPA is a practical and rapid test that can be applied in a clinical setting. The IPA may be a valuable functional outcome measure for clinical trials, complementing structural retinal OCT data in a biological meaningful way. The IPA should be further investigated for suitability for optic neuritis treatment trials.

Quantification of RAPD by an automated pupillometer in asymmetric glaucoma and its correlation with manual pupillary assessment.

PURPOSE: The relative afferent pupillary defect (RAPD) is an important sign of asymmetrical retinal ganglion cell damage. The purpose of this study was to quantify RAPD by a pupillometer (RAPiDo, Neuroptics) and assess its correlation with asymmetric glaucoma and manual pupillary assessment. METHODS: A total of 173 subjects were enrolled in the study and categorized into glaucoma, n = 130, and control, n = 43. Subjects were all recruited in the Glaucoma Clinic of the Aravind Eye Hospital in Madurai during their follow-up. They were 18 years and older, with best corrected visual acuity of 6/36 or better. Exclusion criteria included all retinal pathologies, optic atrophies, ocular injuries, severe uveitis, cloudy corneas, dense cataracts, or use of mydriatics or miotic drugs. RAPD was assessed in all subjects using an automated pupillometer and the results were compared with the swinging flash light test conducted on the same subjects by an experienced ophthalmologist. We looked at the correlation between RAPD and the intereye difference in cup-to-disc ratio (CDR), mean deviation (MD) of visual field testing, and retinal nerve fiber layer (RNFL) thickness. Sensitivity and specificity were assessed by area under the receiver operator characteristic (AUROC) analysis. RESULTS: Glaucoma patients had significant RAPD (0.55 ± 0.05 log units) when compared with the controls (0.25 ± 0.05 log units), P < 0.001. Significant intereye differences in CDR, MD, and RNFL between glaucoma and control (P < 0.001) were seen. There was a good correlation between the magnitude and sign of RAPD and these intereye differences in CDR (r = 0.52, P < 0.001), MD (r = 0.44, P < 0.001) and RNFL thickness (r = 0.59, P < 0.001). When compared with the experienced ophthalmologist, AUROC was 0.94, with 89% sensitivity and 91.7% specificity. CONCLUSION: The good correlation between the magnitude of RAPD, as measured by the automated pupillometer, and intereye differences in MD, CDR, and RNFL thickness in glaucomatous, and the good sensitivity and specificity when compared with the experienced ophthalmologist, suggest that pupillometry may be useful as a screening tool to assess asymmetric glaucoma.

Neurological Assessment.

Reasons for completing a neurological exam include: detecting life-threatening conditions, identifying nervous system dysfunction and the effects of this dysfunction on activities of daily living, comparing current data to previous exams to determine trends, and to provide a database upon which to base collaborative care across disciplines. In this third article of a four-part series, subjective and objective assessment of the neurological exam is reviewed.

Kinematic and kinetic analysis of the inter- and intra-applicator assessment of the Babinski reflex.

AIMS OF THE STUDY: The first aim was to quantify variability in the mechanical technique used by neurologists to elicit the Babinski reflex. The second aim of the study was to assess if the mechanical technique is an important determinant of the subsequent reflex response. MATERIALS AND METHODS: In this study, twelve neurologists elicited the Babinski reflex five times on the same foot of the same participant using a special reflex hammer which recorded the force and duration of the stroke. Hallux movement, tibialis anterior maximum EMG amplitude and pain felt by the participant for each stroke were recorded. RESULTS: A large inter- and intra-applicator variability was shown amongst the neurologists. The change in hallux angle was significantly correlated with the duration of the stroke (R(2)=0.18, P<0.01), maximum (R(2)=0.14, P=0.01) and average (R(2)=0.17, P<0.01) force used to elicit the reflex. No correlations were shown between the hammer forces and duration and the maximum amplitude of the tibialis anterior. Significant correlations were shown between the pain score and the maximum (R(2)=0.15, P<0.01) and average (R(2)=0.17, P=0.001) force used to elicit the Babinski reflex. CONCLUSION: These results indicate that there was substantial variation when performing the Babinski reflex test within and between neurologists which could lead to differences in the resultant reflex and therefore may affect subsequent diagnoses.

Dysphagia risk assessment in acute left-hemispheric middle cerebral artery stroke.

BACKGROUND AND PURPOSE: Bedside evaluation of dysphagia may be challenging in left middle cerebral artery (MCA) stroke due to frequently existing aphasia. Here we analyse the predictive value of common bedside screening tests and of two items of cortical dysfunction, aphasia and buccofacial apraxia (BFA), for the detection of dysphagia. METHODS: We prospectively examined 67 consecutive patients with clinical and imaging evidence of acute (<72 h) left MCA stroke. Dysphonia, dysarthria, abnormal volitional cough and abnormal gag reflex were assessed followed by a standardized 50-ml water-swallowing test determining the symptoms cough and voice change after swallow. Aphasia and BFA were assessed according to defined criteria. Fibre-optic endoscopic evaluation of swallowing (FEES) was performed for validation of dysphagia. RESULTS: 41 (61%) patients had FEES-proven dysphagia. Abnormal gag reflex, abnormal volitional cough, cough after swallow, aphasia and BFA were significantly more frequent in dysphagic as compared to non-dysphagic patients, while dysphonia, dysarthria and voice change after swallow were not. Aphasia and BFA had the highest sensitivity (97 and 78%, respectively) and high negative predictive values (89 and 68%, respectively) for dysphagia. Multivariate regression analysis did not identify an independent predictor of dysphagia. CONCLUSIONS: In left MCA stroke, the sensitivity and specificity of common bedside dysphagia screening methods are low. In contrast, aphasia and BFA have a high sensitivity and high negative predictive power, presumably due to the neuro-anatomical overlap between cortical regions involved in swallowing, speech production, imitation and voluntary movement control.

Evaluation of diabetic foot screening in Primary Care.

AIM: To ascertain whether patients with type 2 diabetes are screened for diabetic foot, and to analyze the factors related to patients and centers associated to performance of such screening. MATERIAL AND METHODS: A multicenter, epidemiological, cross-sectional study was conducted. The clinical records of 443 patients with type 2 diabetes monitored at Primary Care for at least 12 months were reviewed. Demographic and healthcare variables and characteristics of the primary care center were recorded. RESULTS: In the previous year, 51.2% of patients had been trained on foot self-care, 56.4% had undergone foot inspection, 39.5% had been examined with a monofilament, and palpation of peripheral pulses and measurement of the ankle-brachial index were performed in 45.8 and 10.1% of patients, respectively. Diabetic foot screening (inspection, monofilament testing, and palpation of peripheral pulses) was performed in 37% of study patients. Ulcer risk stratification was done in 12.4% of patients. A significant association was found between diabetic foot screening and presence of foot deformities (P<.001), history of neuropathy (P=.005), and history of peripheral artery disease (P<.05). Screening was also associated to some characteristics of the center, such as reception of information about goal achievement (P<.001) and economic incentives for goal attainment (P<.001). CONCLUSIONS: Compliance with diabetic foot screening and ulcer risk stratification in patients with type 2 diabetes in Primary Care was poor.

Standard operating procedures for neurophysiologic assessment of male sexual dysfunction.

INTRODUCTION: Can neurophysiological testing in male patients with sexual dysfunction benefit the decision-making process? The answer remains unclear. AIM: To provide standard operating procedures for the neurophysiologic assessment of male sexual dysfunction. METHODS: Medical literature was reviewed and combined with expert opinion of the authors. RESULTS: Bulbocavernosus reflex latency time, pudendal somatosensory evoked potentials, and sympathetic skin responses have been considered as potential candidates for the diagnosis and assessment of erectile dysfunction (ED). Currently, there is no consensus on a standardized methodology for these neurophysiological investigations in the overall assessment of ED. These procedures are unable to assess the integrity of the efferent parasympathetic proerectile penile innervation; accordingly, none of these assessment procedures is recommended for ED patients. Corpus cavernosum electromyography (CC-EMG) can detect abnormalities in cavernous smooth muscle although these alterations can be attributed both to damage to autonomic penile innervation and to degenerative processes of the cavernous smooth muscle. CC-EMG is still considered experimental. Evidence does not support that men with premature ejaculation (PE) are consistently characterized by penile hypersensitivity; accordingly, penile threshold determination is not recommended to in the diagnosis of PE. Neurophysiological investigation of other components of the penile sensory pathways in PE patients has not provided any definitive contribution to the diagnosis. CONCLUSION: No neurophysiological assessment procedures yield additional information that consistently aids in the assessment of PE and ED.

[Epidemiology and clinical assessment of spasticity in multiple sclerosis]. / Épidémiologie et évaluation clinique de la spasticité dans la sclérose en plaques.

Spasticity is a commonly seen symptom in patients with multiple sclerosis (MS). The vast majority of patients will suffer from this symptom during the course of the disease, and one- third of patients considers that spasticity contributes to a greater part of their disability. The symptom is frequently disabling. It can, however, allow some activities to be performed. Treatment of the symptom is sometimes deleterious, which is why strict assessment of the consequences of spasticity and anticipation of the outcome of antispastic treatment are necessary. Clinical scales, such as the Ashworth and Tardieu scales, are used in clinical practice. The essential element is not, however, assessment of the symptom, but its repercussions on activities of everyday life. It is important to make a list of what patients consider to be disabling situations to verify that they are truly consequences of spasticity. Considering the heterogeneity of clinical expression of spasticity in patients with MS, the use of a scale such as goal attainment scaling (GAS) can probably be totally adapted for the assessment of the effects of antispastic treatment.

Simple foot tapping test as a quantitative objective assessment of cervical myelopathy.

STUDY DESIGN: A clinical and cohort study. OBJECTIVE: The first purpose of this study was to investigate the standard value of a simple foot tapping test (FTT) in a large healthy population. The second purpose was to elucidate the validity of FTT as a quantitative assessment of lower extremity motor function for cervical compressive myelopathy. SUMMARY OF BACKGROUND DATA: Several clinical performance tests have been reported as objective assessments for the severity of cervical myelopathy. The FTT is the simplest and easiest method for a quantitative analysis of lower limb motor dysfunction in the upper motor neuron diseases. However, there were few studies about the FTT in cervical myelopathy. METHODS: We recruited 252 patients who were diagnosed with cervical myelopathy and 792 healthy volunteers who participated in a health promotion project. Among the patients, 126 who underwent surgery were evaluated both before and 1 year after surgery. We performed the FTT and grip and release test and evaluated the modified Japanese Orthopaedic Association (JOA) score for cervical myelopathy. RESULTS: The mean value of FTT was 23.8 ± 7.2 in myelopathic patients, which was significantly lower than 31.7 ± 6.4 in healthy controls and decreased with age. The value of FTT significantly correlated with the lower extremity motor function of modified JOA score and the value of grip and release test. Among the patients who underwent surgery, the average value of FTT was 22.4 ± 7.0 preoperatively and improved to 28.4 ± 8.1 at 1 year postoperatively. Postoperative gain of FTT significantly correlated with the gain of JOA score. CONCLUSION: The FTT results correlated with those of other tests for cervical myelopathy, and the FTT scores were improved by surgery. The FTT is an easy and useful quantitative assessment method for lower extremity motor function in patients with cervical myelopathy, especially those who cannot walk.

Long term clinical and electrophysiological assessment of Croatian children with corticospinal tract involvement in Guillain-Barré syndrome (GBS).

Guillain-Barré syndrome (GBS) is characterized by areflexia. Hyperreflexia is reported in acute motor axonal neuropathy (AMAN). We present 16 children with GBS at the age of 14 months to 13 years. All children studied fulfilled accepted diagnostic criteria for GBS. Hyperreflexia or positive Babinski sign were obtained in all children studied during follow up. Brain and spinal cord MR scans did not reveal any significant structural and morphological abnormalities of central nervous system. The children were examined clinically and electromyoneurographically 2-5 times successively during 1-8.5 years of follow-up. According to established electrodiagnostic criteria demyelinating form of GBS was most common (68%) compared to axonal (18,7%) or mixed form (12,5%). No children had antecendent Campylobacter jejuni infection. Antiganglioside antibodies were detected in 18,7% of patients associated with demyelinating or mixed (axonal/demyelinating) form. Time to nadir and recovery period of walking ability is prolonged more often in demyelinating GBS. Clinical improvement occur earlier compared to improvement of abnormal electrophysiological parameters.Outcome was excellent in 11 in the period 1 month-8.5 years. Hyperreflexia usually appeared in recovery period suggesting involvement of upper motor neurons or spinal interneurons occurring in Croatian children with both demyelinating and axonal form of GBS usually associated with milder course of disease.

[The Sensory Modality Assessment and Rehabilitation Technique (SMART): a behavioral assessment scale for disorders of consciousness]. / La Sensory Modality Assessment and Rehabilitation Technique (SMART) : une échelle comportementale d'évaluation et de revalidation pour des états altérés de conscience.

INTRODUCTION: Difficulties in detecting bedside signs of consciousness in non-communicative patients still lead to a high rate of misdiagnosis illustrating the need to employ standardized behavioral assessment scales. STATE OF ART: The Sensory Modality Assessment and Rehabilitation Technique (SMART) is a behavioral assessment scale of consciousness that assesses responses to multimodal sensory stimulation in disorders of consciousness. These stimulations can also be considered to have therapeutic value. PERSPECTIVES: We here review the different components and use of the SMART assessment and discuss its validity, reliability, and robustness in clinical practice. The scale has a high intra- and inter-observer reliability thanks to a detailed procedure description. However, in the absence of objective gold standards in the assessment of consciousness, it is currently difficult to make strong claims about its validity. A comparison between SMART and other standardized and validated coma-scales is proposed. CONCLUSION: In our view, SMART is an interesting tool for monitoring patients with altered states of consciousness subsequent to coma. Currently, we await studies on its concurrent validity as compared to other validated behavioral assessment scales and on the effect of SMART stimulations on patient outcome.

Laryngeal sensory testing in the assessment of patients with laryngopharyngeal reflux.

Laryngopharyngeal reflux is commonly encountered in the ENT out-patient setting. It leads to impaired sensory capacity of the laryngeal mucosa. The sensory integrity of the laryngopharynx can be evaluated through endoscopic administration of pulsed air, which stimulates the laryngeal adductor reflex. The pressure of air needed to elicit this reflex indicates the degree of sensory impairment. Such laryngeal sensory testing gives a quantifiable means of assessment in patients with laryngopharyngeal reflux, and can be used to measure the response to treatment. Laryngeal sensory testing is safe and well tolerated by patients.

Assessment of vestibular function of infants and children with congenital and acquired deafness using the ice-water caloric test, rotational chair test and vestibular-evoked myogenic potential recording.

CONCLUSIONS: The vestibular function can be assessed by ice-water caloric test, rotational chair test and VEMP recording in severely hearing impaired infants and young children, and 85% of these patients showed abnormal responses in these tests. OBJECTIVES: To evaluate the vestibular function of infants and young children with congenital and acquired deafness, we examined the semicircular canal and otolith function in their early childhood. MATERIALS AND METHODS: Our subjects were 20 children (11 boys, 9 girls; age range 31-97 months, mean age 54.2 months) with severe hearing impairment. Their vestibular functions were assessed by the ice-water caloric test, rotational chair test and vestibular-evoked myogenic potential (VEMP) recording. RESULTS: Among these 20 severely hearing impaired children, only 3 (15%) showed normal responses in the caloric test, rotational chair test and VEMP recording bilaterally. Seven (35%) showed responses asymmetrically in the caloric test despite normal responses in the rotational chair test and VEMP recording bilaterally. Five (25%) showed hyporeflexia or areflexia in the caloric test bilaterally, but showed normal responses in the rotational chair test and normal reproducible or decreased VEMPs. Five (25%) showed no responses at all in the caloric test, rotational chair test and VEMP recording.

Prenatal organophosphate metabolite and organochlorine levels and performance on the Brazelton Neonatal Behavioral Assessment Scale in a multiethnic pregnancy cohort.

Prenatal exposures to organophosphate pesticides and polychlorinated biphenyls have been associated with abnormal neonatal behavior and/or primitive reflexes. In 1998-2002, the Mount Sinai Children's Environmental Health Center (New York City) investigated the effects of indoor pesticide use and exposure to polychlorinated biphenyls on pregnancy outcome and child neurodevelopment in an inner-city multiethnic cohort. The Brazelton Neonatal Behavioral Assessment Scale was administered before hospital discharge (n = 311). Maternal urine samples were analyzed for six dialkylphosphate metabolites and malathion dicarboxylic acid. A random subset of maternal peripheral blood samples from the entire cohort (n = 194) was analyzed for polychlorinated biphenyls and 1,1'-dichloro-2,2'-bis(4-chlorophenyl)ethylene. Malathion dicarboxylic acid levels above the limit of detection were associated with a 2.24-fold increase in the number of abnormal reflexes (95% confidence interval: 1.55, 3.24). Likewise, higher levels of total diethylphosphates and total dialkylphosphates were associated with an increase in abnormal reflexes, as was total dimethylphosphates after paraoxonase expression was considered. No adverse associations were found with polychlorinated biphenyl or 1,1'-dichloro-2,2'-bis(4-chlorophenyl)ethylene levels and any behavior. The authors uncovered additional evidence that prenatal levels of organophosphate pesticide metabolites are associated with anomalies in primitive reflexes, which are a critical marker of neurologic integrity.