Abnormal Deep Tendon Reflexes Research Articles

Pattern and implications of neurological examination findings in autosomal dominant Alzheimer disease.

As knowledge about neurological examination findings in autosomal dominant Alzheimer disease (ADAD) is incomplete, we aimed to determine the frequency and significance of neurological examination findings in ADAD. Frequencies of neurological examination findings were compared between symptomatic mutation carriers and non mutation carriers from the Dominantly Inherited Alzheimer Network (DIAN) to define AD neurological examination findings. AD neurological examination findings were analyzed regarding frequency, association with and predictive value regarding cognitive decline, and association with brain atrophy in symptomatic mutation carriers. AD neurological examination findings included abnormal deep tendon reflexes, gait disturbance, pathological cranial nerve examination findings, tremor, abnormal finger to nose and heel to shin testing, and compromised motor strength. The frequency of AD neurological examination findings was 65.1%. Cross-sectionally, mutation carriers with AD neurological examination findings showed a more than two-fold faster cognitive decline and had greater parieto-temporal atrophy, including hippocampal atrophy. Longitudinally, AD neurological examination findings predicted a significantly greater decline over time. ADAD features a distinct pattern of neurological examination findings that is useful to estimate prognosis and may inform clinical care and therapeutic trial designs.

Investigating the Effect of Zinc on the Prevention of Acute Peripheral Neuropathy in Cancer Patients Treated with Taxanes.

Background:Chemotherapy-induced peripheral neuropathy (CIPN) is a major complication of many chemotherapeutic agents, including taxanes. Here, we aimed to investigate the effect of zinc on CIPN.Materials and Methods:This is a double-blinded controlled clinical trial that was performed in 2020–2021 in Isfahan on 55 cancer patients. We collected the data regarding CIPN, its severity, presence of abnormal deep-tendon reflexes, paresthesia, restriction in daily activities, and restriction in self-care and pain. Patients were divided into two groups: Patients in the first group were treated with capsules of zinc sulfate 25 mg daily and the control group received placebo. The duration of treatments was 3 months. Patients were visited 6, 9, and 12 weeks after study initiation.Results:There was a statistically significant decrease in the frequency of CIPN in the intervention group (37.03% vs. 14.8%, P < 0.001). The evaluation of the severity of neuropathy and presence of abnormal deep-tendon reflexes also demonstrated significant decrease in the intervention group during the study (P < 0.001 for both), but no significant changes were observed in the placebo group (P > 0.05). The activity limitations and pain severity improved significantly both in the intervention and placebo groups (P < 0.001 for both groups and items). The intervention group, however, had significantly lower frequencies of activity limitation and lower pain severity within compared to the control group during the study (P < 0.001).Conclusion:Zinc supplement therapy resulted in reduced frequency and intensity of CIPN in patients undergoing chemotherapy with taxanes.

Early progression of Krabbe disease in patients with symptom onset between 0 and 5\u2009months

BackgroundKrabbe disease is a rare neurological disorder caused by a deficiency in the lysosomal enzyme, β-galactocerebrosidase, resulting in demyelination of the central and peripheral nervous systems. If left without treatment, Krabbe disease results in progressive neurodegeneration with reduced quality of life and early death. The purpose of this prospective study was to describe the natural progression of early onset Krabbe disease in a large cohort of patients.MethodsPatients with early onset Krabbe disease were prospectively evaluated between 1999 and 2018. Data sources included diagnostic testing, parent questionnaires, standardized multidisciplinary neurodevelopmental assessments, and neuroradiological and neurophysiological tests.ResultsWe evaluated 88 children with onset between 0 and 5 months. Median age of symptom onset was 4 months; median time to diagnosis after onset was 3 months. The most common initial symptoms were irritability, feeding difficulties, appendicular spasticity, and developmental delay. Other prevalent symptoms included axial hypotonia, abnormal deep tendon reflexes, constipation, abnormal pupillary response, scoliosis, loss of head control, and dysautonomia. Results of nerve conduction studies showed that 100% of patients developed peripheral neuropathy by 6 months of age. Median galactocerebrosidase enzyme activity was 0.05 nmol/h/mg protein. The median survival was 2 years.ConclusionsThis is the largest prospective natural history study of Krabbe disease. It provides a comprehensive description of the disease during the first 2 years of life. With recent inclusion of state mandated newborn screening programs and promising therapeutic interventions, enhancing our understanding of disease progression in early onset Krabbe disease will be critical for developing treatments, designing clinical trials, and evaluating outcomes.

The clinical features of combined central and peripheral demyelination in Chinese patients

BackgroundCombined central and peripheral demyelination (CCPD) is rare and has never been reported as a spectrum disease in Han Chinese population. ObjectivesTo study the clinical features of CCPD in Han Chinese patients. MethodsTwenty-two CCPD patients were selected from 788 demyelination cases. We reviewed and compared the clinical manifestation, laboratory data, electrophysiological examination, MRI and the prognosis. ResultsCCPD patients presented with sensory disturbance (86.4%), plegia (77.3%), cranial nerve involvement (77.3%), abnormal deep tendon reflexes (72.7%). CSF data showed increased CSF protein in 81% patients. Oligoclonal IgG bands (OB) were negative. Cortical or juxtacortical, periventricular, infratentorial lesions, thoracic and cervical spinal cord were mostly affected. Visual evoked potentials indicated optic nerves demyelinating in 50% cases. 21 CCPD patients were treated with intravenous immunoglobulin or steroids or both of them, and the efficacy was 33.3%, 54.5%, 71.4%, respectively. One case that showed no response to steroids plus intravenous immunoglobulin treatment was improved significantly after using cyclophosphamide. ConclusionsCCPD is a spectrum disease that can't be regarded as a simple combination of MS and CIDP. A suspected CCPD should receive brain and spinal MRI as well as electrophysiological examination to obtain a precise diagnosis.

Neurological disorders in HIV-infected children in India

There are few studies of HIV-related neurological disorders from centres in low-income countries where facilities are available for detailed investigation. Records of all patients attending the paediatric HIV outpatient department at B. J. Wadia Hospital for Children, Mumbai between April 2000 and March 2008 were reviewed. Of 668 HIV-infected patients, 48 (7.2%) had neurological manifestations and are included in this study. Twenty-six (54.2%) children had HIV encephalopathy. Other causes of neurological manifestations include febrile convulsion in five (10.4%), bacterial meningitis in three (6.3%), epilepsy in two (4.2%), tuberculous meningitis and progressive multi-focal encephalopathy in two (4.2%) each and toxoplasmosis, vasculitis, acute demyelinating encephalomyelitis, anti-phospholipid antibody syndrome, Down's syndrome, birth asphyxia, herpes simplex encephalopathy and mitochondrial encephalopathy in one (2.1%) each. Mean (SD) age at presentation was 4.36 (3.38) years with a range of 2 months to 15 years. The common subtle neurological manifestations were abnormal deep tendon reflexes and extensor plantar reflexes. The common symptomatic manifestations were delayed milestones in 21 children (43.8%) and seizures in 19 (39.6%). Seizures were more common in males (54%) than in females (25%) (p=0.038). In children <5 years, delayed milestones was the most common manifestation while focal neurological deficits were more common in older children. Of the 13 children who received HAART, nine (60.23%) improved. Early diagnosis of neurological disorders in HIV-infected children is important for appropriate investigation and management, especially the introduction of HAART.

Predictors of long term neurological outcome in bacterial meningitis

To study the long-term neurological and developmental outcome and the clinical and laboratory predictors of sequelae in children with acute bacterial meningitis (ABM). Detailed clinical and demographic data was retrieved from the medical records of study children. Subsequently they were followed up for a minimum of 12 months after discharge for development, neurological and hearing assessment. All sequelae were identified and divided into minor or major. For analysis data was divided into 2 groups those with sequelae and without sequelae at follow-up. Statistical analysis was done using SPSS version 10.00 and Epi Info version 2000. 61 boys and 19 girls, a mean age of 31.4 +/= 41.9 months at the time of ABM, were included in the study. Of these 62.5% children were infants. Mean age at follow-up was 58.6 +/= 47.2 months. Sequelae were observed in 32 (40%) children (8 (10%) minor and 24 (30%) major). Mean social quotient at follow-up was 92.8 +/= 32.6. Developmentally 22 (37.9%) children were normal and 20 (34.5%) had global delay. Seizures (P=0.015), cranial nerve palsy (P=0.0065), abnormal deep tendon reflexes (P=0.002), Glasgow coma scale score (GCS) < 8 (P = 0.044) at admission, a CSF culture positive for bacteria and abnormal findings on ultrasonography or computed tomography of head at admission had significant association with sequelae at follow-up. All children (7/7) who had infarct on CT scan (P=0.001) and 12 (80%) of 15 patients who had hydrocephalus (OR - 9.0, 95% CI - 2.03-45.6, P=0.001) diagnosed on CT scan developed severe sequelae. On multiple regressions GCS score <8, presence of cranial nerve palsy and abnormal deep tendon reflexes were independent predictors of sequelae. Neurological and audiological sequelae and global developmental delay may be seen in about one third of survivors of bacterial meningitis. GCS score <8, presence of infarct or cranial nerve palsy, or hydrocephalous on CT/ ultrasound at admission may help in identification of children most likely to need long term follow up and rehabilitation.

Magnetic Resonance Imaging Abnormalities of Neural Axis in Lenke Type 1 Idiopathic Scoliosis

Patients with Lenke type 1 single thoracic idiopathic scoliosis were included in this prospective study. All patients had preoperative magnetic resonance imaging (MRI). To examine the frequency of neural axis abnormalities and the need for preoperative MRI in this group of patients. Because of the increasing use of MRI, neural axis abnormalities have been reported in association with certain clinical and radiologic findings in idiopathic scoliosis cases. A total of 104 patients (49 juvenile onset and 55 adolescent onset), older than 10 years, were included in the study. The association of neural axis abnormalities with pes cavus, abnormal deep tendon reflexes, age of onset, presence of pain, severity of the frontal plane deformity, and sagittal contours were investigated. All 7 patients with a neural axis abnormality on MRI had an early onset disease, and 6 of them had back pain. Thus, age of onset and back pain seem to be predictive of these abnormalities. Frequency of MRI abnormalities was as high as 45% for patients with back pain in addition to a type IC curve. In patients with juvenile idiopathic scoliosis and back pain, preoperative MRI should be performed to eliminate the risk of postoperative neurologic deficits, even if the scoliosis is Lenke type 1. However, in patients with Lenke type 1 idiopathic scoliosis, preoperative MRI studies seem unnecessary if intraoperative neural monitoring is to be performed.

Evaluation of Spasticity in Adults

Spasticity is one component of the upper motor neuron syndrome, which also includes motor deficits and loss of fine movement. Of these three components of the upper motor neuron syndrome, only spasticity is amenable to treatment, hence, the interest in this symptom. Evaluation of spasticity must therefore consider the patient as a whole. The patient presents a disorder of somatic motility that must be described precisely. Clinical examination must identify signs suggestive of spasticity such as alteration of passive mobilization of the limb segment, and abnormal deep tendon reflexes. Electrophysiology may help to define and quantify the altered reflexes. The most important step then consists of demonstrating that the spasticity, its consequences, or both, interfere with the patient’s natural movement (or with the patient’s remaining functional possibilities when voluntary movement is totally abolished), an essential prerequisite to determining a treatment program. Reversible tests (motor blocks, intrathecal baclofen test injections) provide a major contribution to the determination of functional impairment caused by spasticity and allow the objectives of treatment to be clearly defined.

Neurological features of cerebral malaria in Nigerian children.

Cerebral malaria is one of the commonest causes of an acute neurological syndrome in malaria-endemic areas. However, there are few detailed reports of findings on clinical neurological examination of the condition. The neurological features of cerebral malaria in 103 children aged 5 years or less were studied in Ibadan, Nigeria, an area of high malaria transmission. The correlation of these features with prognosis was also studied. Convulsions occurred in 87% of subjects and were in most cases of a generalized tonic-clinic nature. Abnormalities of posture were observed in 41%, abnormal tone in 70% and abnormal deep tendon reflexes in 74%. Absent corneal reflexes were found in about 14%. The time interval between the last seizure episode and presentation in hospital, abnormal posture (decerebrate or decorticate), absence of corneal reflex and depth and duration of coma were indicators of poor prognosis. In this study, cerebral malaria presented with non-specific features of diffuse, symmetrical, upper motor neurone dysfunction, and some specific neurological features were associated with poor prognosis. It is important that cerebral malaria be considered in any child with features of acute encephalopathy in a malaria-endemic area. Careful clinical examination of such children is essential as neurological features of the condition may provide a clue to prognosis.

ASSESSMENT OF PERIPHERAL NERVE FUNCTION IN AN ECUADORIAN RURAL POPULATION EXPOSED TO PESTICIDES

To explore the peripheral nervous system effects of regular agricultural pesticide use, a cross-sectional survey was conducted in highland Ecuador. Participants were 144 occupationally exposed farm members, 30 female farm members with little direct exposure, and 72 unexposed local town residents, frequency matched to the exposed people on age, sex, and education. Organophosphorus and carbamate insecticides and dithiocarbamate fungicides accounted for the majority of pesticide applications, with leaking backpack sprayers, minimal use of personal protective equipment, and frequent dermal contact being the norm. In polytomous logistic regression analyses, applicators had significantly greater odds for more current peripheral nerve symptoms (odds ratio OR = 3.1), signs of poor coordination (OR = 4.3), abnormal deep tendon reflexes (OR = 2.9), and reduced power (OR = 2.1) compared to controls. Mean toe vibration threshold scores, on a logarithmic scale, were significantly higher among applicators (beta= 0.035) and those reporting previous pesticide poisonings (beta= 0.074). Such indicators of peripheral nervous system effects may be due to a variety of factors, including high pesticide exposure conditions.

Development of Subtle Neurological Signs after Systemic Illness in HIV-infected Individuals

Thirty human immunodeficiency virus (HIV) infected individuals entered a longitudinal study without signs of dysfunction of the central nervous system (CNS). Nine of these individuals developed a systemic illness between study visits, and 7 of these 9 patients (78%) had neurological signs at the next examination (e.g., action-intention tremors, abnormal gait, release signs, abnormal deep tendon reflexes). Only 2/21 (9.5%) of the subjects who did not develop systemic illness showed such signs. These data are consistent with the hypothesis that other factors (e.g. cytokines) as well as the HIV may cause subtle CNS dysfunction.

Neurologic History and Examination Results and Their Relationship to Human Immunodeficiency Virus Type 1 Serostatus in Hemophilic Subjects: Results From the Hemophilia Growth and Development Study

In a prospective study of the growth and neuropsychologic function of hemophilic subjects, 333 boys, median age of 12.3 years, had baseline neurologic examinations. The study population included 207 individuals (62%) who were seropositive for human immunodeficiency virus type 1 (HIV-1). Overall results indicated that 11% had abnormalities of cranial nerve function, 17% had abnormal deep tendon reflexes, 23% had abnormal strength, 25% had abnormal coordination, and 31% had abnormal tone, bulk, or range of motion. By contrast, 2% or fewer displayed abnormal movements or had abnormal pain or vibratory sensation, or altered mental status. Abnormalities were more common in older hemophilic subjects (eg, 67 [38%] of 177 subjects > or = 12 years of age had abnormal tone, bulk, or range of motion vs 36 [23%] of 156 subjects < 12 years of age). When compared with regard to HIV-1 status, HIV-seronegative and HIV-seropositive subjects did not differ with regard to head circumference or the frequency of abnormalities of cranial nerve function, sensation, muscle strength, or coordination. However, deep tendon reflexes and tone, bulk, or range of motion were more frequently abnormal in HIV-1-seropositive individuals. More HIV-1-positive subjects had at least one increased deep tendon reflex (13/207 [6.3%] vs 1/126 [0.8%] in seronegatives) and more had non-hemophilia-related decreases in muscle bulk (7/207 [3.4%] vs 0/126 in seronegatives). These results indicate that hemophilia causes substantial neurologic dysfunction and that certain findings, such as changes in muscle-stretch reflexes or muscle bulk, may also reflect the neurologic consequences of HIV infection.

Neuropsychological effects of chemotherapeutic agents

This study examines the neuropsychological effects of intrathecal and intravenous chemotherapies, specifies the relationship between drug administrations and test performance, and addresses differential effects of chemotherapy related to treatment age. Participants were 19 children under 6 years of age who were receiving intrathecal and intravenous chemotherapy for the treatment of acute lymphocytic leukemia (n = 15) or Burkitt's lymphoma (n = 4). Neuropsychological test performances obtained within 3 months of diagnosis and 1 year later were compared. Stability of performance was indicated on measures of higher order cognitive abilities and gross‐motor skills. Deficits in fine‐motor skills and abnormal deep tendon reflexes observed at baseline showed significant improvement 1 year later. Recovery was associated with discontinuation of systemic vincristine and attributed to rebound from chemotherapy‐induced peripheral neuropathy.

TOOTH RING ANALYSIS IN CEREBRAL PALSY.

Introduction Infantile cerebral palsy consists of syndromes characterized by chronic motor disabilities due to brain involvement, occurring primarily in the prenatal or perinatal period. It may be classified into three general subgroups 1,2 : (1) spastics , characterized clinically by the presence of increased stretch reflexes in the muscles and by increased deep tendon reflexes; (2) dyskinesias , characterized by abnormalities in motion without abnormal deep tendon reflexes—the most common form of dyskinesia is the athetoid; (3) ataxias , in which dyssynergia and poor balance are characteristic. The spastics and athetoids make up roughly 90% of the total cerebral palsy population. The causes of cerebral palsy are basically of two types: (1) anoxia,* which tends to be associated with brain lesions resulting in the clinical syndromes of dyskinesia, primarily athetosis, and (2) trauma and vascular damage, which tend to be associated with the clinical syndromes of spasticity. The correlation between specific syndromes and etiologies