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PubMed2006

Magnetic resonance imaging abnormalities of neural axis in Lenke type 1 idiopathic scoliosis.

STUDY DESIGN: Patients with Lenke type 1 single thoracic idiopathic scoliosis were included in this prospective study. All patients had preoperative magnetic resonance imaging (MRI). OBJECTIVE: To examine the frequency of neural axis abnormalities and the need for preoperative MRI in this group of patients. SUMMARY OF BACKGROUND DATA: 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. METHODS: 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. RESULTS: 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. CONCLUSION: 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.

Adolescent↗
PubMed2006

The treatment of disc-herniation-induced sciatica with infliximab: one-year follow-up results of FIRST II, a randomized controlled trial.

STUDY DESIGN: A randomized controlled trial. OBJECTIVES: To evaluate the long-term efficacy of infliximab, a monoclonal antibody against tumor necrosis factor alpha (TNF-alpha), in patients with acute/subacute sciatica secondary to herniated disc. SUMMARY OF BACKGROUND DATA: The results of experimental studies and our open-label trial support the use of infliximab in sciatica. Here we report the 1-year results of a randomized controlled trial (FIRST II, Finnish Infliximab Related STudy) evaluating the efficacy and safety of a single infusion of infliximab for sciatic pain. METHODS: Inclusion criteria were unilateral sciatic pain with a disc herniation concordant with the symptoms and signs of radicular pain. Patients had to be candidates for discectomy. Criteria for discectomy included (in addition to a symptomatic disc herniation on MRI) neural entrapment (straight leg raising [SLR] < or =60 degrees ) with either a short-term (2-4 weeks) severe or long-term (4-12 weeks) moderate leg pain. Forty patients were allocated to a single intravenous infusion of either infliximab 5 mg/kg or placebo. Differences in the clinical examination parameters (straight leg raise [SLR], muscle strength, sensory defects, tendon reflexes), patient-reported symptoms (leg and back pain using a visual analog scale [VAS], Oswestry disability, quality-of-life [RAND-36]), sick leaves, number of discectomies, and adverse effects between the two treatment groups over the 1-year follow-up were compared using Mann-Whitney U test or Student's t test, repeated-measures analysis, or Cox proportional hazards model. Logistic regression was used to assess the predictors of good response. RESULTS: Sixty-seven percent of patients in the infliximab group reported no pain at 52 weeks compared with 63% in the control group (P = 0.72). Similar efficacy was observed between treatment groups for other outcomes. Eight patients in each group required surgery. Three nonserious adverse reactions were encountered in the infliximab group. The response (irrespective of the treatment) was significantly better with shorter symptom duration and less SLR restriction at baseline. Patients in the infliximab group appeared to especially benefit in cases of a L4-L5 (or L3-L4) herniation and if a Modic change was colocalized at the symptomatic level. CONCLUSIONS: Although the long-term results of this randomized trial do not support the use of infliximab compared with placebo for lumbar radicular pain in patients with disc herniation-induced sciatica, further study in a subgroup of patients with L4-L5 or L3-L4 herniations, especially in the presence of Modic changes, appears to be warranted.

Acute Disease↗
PubMed2004

Clinical presentation and anatomic position of L3-L4 disc herniation: a prospective and comparative study.

OBJECTIVE: A prospective, controlled cohort study was conducted to assess the anatomic transverse location and clinical presentation of L3-L4 disc herniation compared with lower lumbar levels. METHODS: This study prospectively identified 37 patients diagnosed with L3-L4 disc herniation (study group) and 52 patients diagnosed with L4-L5 and L5-S1 herniation (control group). The following clinical data were collected: age, femoral stretch test, motor strength, sensation, and deep tendon reflexes. The anatomic transverse location of the disc fragments was assessed by computed tomography or magnetic resonance imaging and was classified as either central, posterolateral, foraminal, or far lateral. RESULTS: The patients in the study group were older than the patients in the control group, and neurologic deficit was more common. The transverse location was foraminal and extraforaminal in 59% of the study group compared with 27% of the control group. These differences were statistically significant. CONCLUSIONS: The incidence of foraminal and far lateral disc herniation is significantly higher at the L3-L4 level compared with lower lumbar levels. When examining an older patient complaining of thigh pain, special attention should be given to the quadriceps strength, patellar reflex, and femoral stretch test. The L3-L4 foraminal and extraforaminal area should be assessed carefully.

Adolescent↗
PubMed2005

Neuromuscular sequelae of critical illness.

PURPOSE OF REVIEW: To investigate the impact of critical illness polyneuropathy and critical illness myopathy on short-term and long-term patient outcome. RECENT FINDINGS: In the acute-care setting, critical illness polyneuropathy and critical illness myopathy are important causes of acute paralysis in critically ill comatose patients, and may cause inappropriately pessimistic prognoses. Duration of weaning from artificial ventilation is 2 to 7 times greater in patients with critical illness polyneuropathy than in patients without critical illness polyneuropathy. After intensive care unit and hospital discharge, many patients diagnosed with critical illness polyneuropathy or critical illness myopathy are reported to complain of profound muscle weakness. Chronic disability was a common finding among them. Complete functional recovery with patients regaining the ability to breathe spontaneously and to walk independently was reported in 180 of 263 patients (68.4%); severe disability with tetraparesis, tetraplegia, or paraplegia was reported in 74 patients (28.1%). Persisting milder disabilities were common even in patients with complete functional recovery, and included reduced or absent deep tendon reflexes, stocking and glove sensory loss, muscle atrophy, painful hyperesthesia, and foot drop. An association of critical illness polyneuropathy and critical illness myopathy with increased intensive care unit and hospital mortality has been demonstrated only in selected intensive care unit populations; data are insufficient to demonstrate any association with long-term mortality. SUMMARY: Intensive care unit-acquired critical illness polyneuropathy and critical illness myopathy influence the evaluation of acutely ill comatose patients and may instigate unreasonably pessimistic prognosis. Critical illness polyneuropathy and critical illness myopathy are an important cause of difficult weaning of patients from the ventilator and of persisting muscle weakness and disability after intensive care unit discharge.

Causality↗
PubMed2005

Effect of penile vibratory stimulation on spasticity in men with spinal cord injury.

OBJECTIVE: Penile vibratory stimulation is the first treatment option for anejaculation in men with spinal cord injury. It has been postulated that it also has an antispasticity effect. The purpose of this study was to determine the effect of penile vibratory stimulation on spasticity in patients with spinal cord injury. DESIGN: Ten male patients with spinal cord injury were included in the study. They were examined at the baseline and 3, 6, 24, and 48 hrs later. After their baseline examination, they performed penile vibratory stimulation. The outcome variables were spasticity (Ashworth scale), spasm frequency, spasm severity, painful spasms, plantar stimulation response, deep tendon reflexes, clonus, and effect on function. RESULTS: Ashworth grade showed a statistically significant decrease at hour 3 and hour 6 examinations (P = 0.001 and P = 0.03, respectively, with Tukey test). The patients showed a tendency toward having less frequent and less severe spasms throughout the study; however, it did not reach to a significant level. Similarly, clonus showed a nonsignificant decrease during follow-up examinations. The other variables did not change considerably. CONCLUSIONS: Penile vibratory stimulation may contribute to the relief of the spasticity in men with spinal cord injury. In treating spasticity, all the factors that increase or decrease the tone should be considered.

Adult↗
PubMed2006

Mitochondrial disorder aggravated by propranolol.

Although there are indications that beta-blockers affect the skeletal muscle in therapeutic dosages, their influence on mitochondrial disorders is unknown. A 52-year-old woman developed double vision, myalgias, muscle cramps, and hip and thigh muscle stiffness. Clinical neurologic examination revealed ptosis, dysarthria, sore neck muscles, weakness and wasting of the thighs, and generally brisk tendon reflexes. Lactate stress testing was significantly abnormal. Needle electromyography was nonspecifically abnormal and myopathic. Muscle biopsy showed mild myopathic changes, target fibers, and a single COX-negative fiber. Probable mitochondrial disorder was diagnosed. The patient had been on 30 mg of propranolol during 7 years for arterial hypertension. Shortly after discontinuation of the drug, her double vision gradually disappeared, myalgias and muscle cramps gradually resolved, and the patient reported an increase in muscle mass on repeated follow-ups. Long-term administration of propranolol may aggravate a mitochondrial disorder. Discontinuation of propranolol may result in a gradual resolution of these adverse reactions.

Adrenergic beta-Antagonists↗
PubMedDate not supplied

Hypothyroidism with subacute pseudomyotonia--an early form of Hoffmann's syndrome? Report of a case.

A 25-year-old man was admitted to the hospital because of painful muscle cramps and action myospams of subacute onset and 6 weeks' duration. No myotonia could be demonstrated objectively and his deep tendon reflexes showed no prolongation of the relaxation phase. Serum creatinine was raised but creatinine clearance was normal. Serum levels of aldose, CPK, ASAT and ALAT were increased but ordinary light microscopy revealed no histological signs of muscle disease in a quadriceps biopsy. ECG showed a prolonged PQ interval and flat T waves in the left precordial leads. Laboratory tests of thyroid function revealed intensive hypothyroidism, and high titers of circulating thyroid antibodies were demonstrated. During 2 1/2 months of thyroid therapy, the muscle symptoms gradually disappeared completely and the patient could return to work. By that time the serum enzymes and the ECG had normalized. Despite the lack of objective signs of myotonia, we consider that the very dominant subjective muscle symptoms, severe enough to prevent the patient from performing his ordinary manual work and completely reversible on thyroid therapy, justify the designation of hypothyroid myopathy. The question is raised whether the case represents an early form of Hoffmann's syndrome.

Adult↗
PubMed2004

Sensory CIDP presenting as cryptogenic sensory polyneuropathy.

The objective of this study was to report that patients with chronic inflammatory demyelinating polyneuropathy (CIDP) can present with a clinical picture of cryptogenic sensory neuropathy. Patients with distal sensory neuropathy and electrodiagnostic studies that are minimally abnormal or consistent with an axonal pathology are usually diagnosed as having cryptogenic sensory neuropathy if no cause for neuropathy can be found. Some of these patients, however, may have sensory CIDP. We reviewed the records of eight patients with CIDP, diagnosed by sural nerve biopsy, who presented with sensory neuropathy and electrodiagnostic studies that were minimally abnormal or revealed changes consistent with axonal neuropathy. All patients reported distal numbness and paresthesias and, on examination, had predominantly large fiber distal sensory loss and normal muscle strength. In most patients, deep tendon reflexes were reduced or absent. Sural nerve biopsies in all patients were consistent with chronic myelinopathy, with quantitative teased fiber analysis revealing segmental remyelination in 13-40% of the fibers. The four patients who received IVIg therapy had improved sensation and gait. Of the remaining four patients, one is being followed, one had spontaneous remission, one was lost to follow-up, and one, with contraindications to therapy, reported disease progression. Sensory CIDP may present as cryptogenic sensory polyneuropathy with normal or axonal electrophysiologic features. Sural nerve biopsy should be considered in patients with progressive, predominantly large fiber sensory neuropathy of otherwise unknown etiology, as they may have sensory CIDP that responds to therapy.

Adult↗
PubMed2005

History of standard scoring, notation, and summation of neuromuscular signs. A current survey and recommendation.

In this article, we trace the history of scoring, notation, and summation of the neuromuscular signs of muscle weakness and decrease of tendon reflexes and sensation. We recommend a standard system to promote consistency in the effort introduced by Mitchell and Lewis to "represent systems and force by their signs." The scoring of neuromuscular signs began with Mitchell and Lewis in the 19th century who used pluses, minuses, and N (for normal) to express the activity of muscle stretch reflexes. Henry Plummer introduced an ordinal scoring approach for muscle weakness, reflex decrease and increase, and sensation loss. In 1919, he and Walter Sheldon and Henry Woltman introduced standard pre-printed examination forms with written instructions for notation and scoring. Robert Lovett, a Boston orthopedist, scored weak muscles of poliomyelitis patients from 2 (mild weakness) to 6 (paralyzed), 1 being normal. Lovett's approach was used, after reversing the order of the grades and decreasing each grade by 1, by a Committee of the Medical Research Council for evaluating return of muscle weakness after nerve injury. Despite dissimilarity to existing reflex and sensation scores and uneven width of grades, this approach was widely adopted for use in neurologic practice. We introduced the Neuropathy Impairment Score using a combination of the Mitchell, Plummer, and Lovett approaches, summing all individual scores of a standard set of neuromuscular examinations. In a non-representative survey of 19 neuromuscular physicians from different countries, we find that there is a considerable variability in the approaches used for grading. Assuming that scoring is useful, we herein suggest (a) impairments should be scored separately from hyperfunction and (b) for the scoring of impairments (muscle weakness, reflex decrease, and sensation loss), the same ordinal scoring approach should be used with 0 as normal and 1, 2, ... representing increasing impairment based on the judgment of percentage abnormality with corrections made for age, sex, physical fitness, and physical characteristics.

Data Collection↗
PubMed2004

Idiopathic unilateral hyperhidrosis with Holmes-Adie syndrome: case report.

Hyperhidrosis is a disease that is characterized by excessive sweating due to hyperactivation of eccrine sweat glands. It may be localized or generalized form. Holmes-Adie syndrome is an idiopathic disease with unilateral pupil dilatation and loss of deep tendon reflexes. We present a 37-year-old female patient diagnosed with unilateral hyperhidrosis coincident with Holmes-Adie syndrome because of this unusual presentation.

Adie Syndrome↗
PubMed1997

Magnesium sulphate for control of spasms in severe tetanus. Can we avoid sedation and artificial ventilation?

A prospective pilot study was undertaken to investigate the ability of magnesium sulphate to control the spasms of severe tetanus without the need for sedation and artificial ventilation. All eight patients admitted with severe tetanus to our intensive care unit within the last year were given magnesium sulphate intravenously as a 5-g loading dose followed by an infusion of 2-3 gh-1. The infusion rate was increased to control spasms while retaining the patella tendon reflex, which proved a valid guide to avoid overdose. Spasms were effectively controlled and serum magnesium concentrations were maintained within the therapeutic range. Spontaneous ventilation was adequate, ventilatory support being required only for the management of lung pathology. There was no evidence of cardiovascular instability due to sympathetic over activity. No supplementary sedation was required for the control of spasms or autonomic dysfunction during magnesium therapy. We conclude that magnesium sulphate can be used as the sole agent for the control of spasms in tetanus without the need for sedation and artificial ventilation.

Adult↗
PubMed1992

The neurologic aspects of transcobalamin II deficiency.

Thirty-four symptomatic cases of inherited transcobalamin II (TCII) deficiency were analysed in order to determine the frequency and nature of neurologic manifestations. In no instance was there definite evidence of a neurologic disorder at the time of presentation as a young infant. One child of 2 1/2 years transiently lost deep tendon reflexes at a time of suboptimal treatment. A syndrome of mental retardation and other neurologic manifestations was observed in three cases, all with the following in common: (1) an extended duration of illness of 2-17 years; (2) inadequate or not treatment with Cbl; (3) treatment with folic of folinic acid. TCII deficiency rarely if ever presents with neurologic manifestations. However, neurologic disorders can be produced subsequently by improper treatment.

Central Nervous System Diseases↗
PubMed1975

Serum triiodothyronine 7-15 years after fractionated low dose radioiodine therapy of thyrotoxicosis.

In 189 of 334 patients, who had been treated with fractionated doses of radioiodine for Graves' disease 7-15 years ago, the serum concentrations of triiodothyronine have been estimated in additition to the following parameters: Protein bound 127-iodine (PB-127-I), free thyroxine index, cholesterol- and TSH-level in serum, tendon reflex time and clinical index according to Billewitz et al. (1969). In forty-one of the 189 sera the free T4 (AFT4) and free T3 (AFT3) concentrations were measured as well. The following hormonal and clinical patterns were observed: (1) Euthyroidism, with all parameters within the normal range in 148 patients (equals 78-4%). (2) Hypothyroidism with low serum T3, PBI, AFT4, AFT3 and elevated TSH six subjects (equals 3-2%). (3) Persistent hyperthyroidism with increased thyroid hormone concentrations and low TSH in seven cases (equals 3-7%). (4) In twenty-eight clinically euthyroid patients (equals 14-8%) TSH was elevated with normal PBI and AFT4. Twenty of these subjects had a low normal T3 and in nine the T3 was clearly in the hypothyroid range (40 plus or minus 12 mug/dl). (5) The constellation of normal T3, low T4 and elevated TSH, which has been frequently found after radioiodine therapy, has been seen in only moderate form in ten cases.

Adult↗
PubMed1982

Measurements of neuromuscular tone and strength in Down's syndrome children.

Twenty-eight Down's syndrome children and 22 normal children were evaluated for patellar tendon reflex, muscle tone and grip strength. All of this subjects were between the ages of 4 and 17 years. It was the purpose of this study to determine the differences between Down's syndrome and normal children. The standard patellar reflex test was used for one measure of muscle tone, and a locally devised muscle palpation test for the second measure of muscle tone. Finally, grip strength was employed as our measure of muscle strength. Results indicated that Down's syndrome children had a less brisk and more irregular patellar reflex response than normal controls. Furthermore, overall muscular tone was less in these Down's syndrome subjects and finally, muscular grip strength was inferior in Down's syndrome children when compared to normal children. Implications for increased physical education and physical activity are noted for this special population.

Adolescent↗
PubMed1984

Plantar ulcers in hereditary sensory neuropathy. A plea for conservative treatment.

Hereditary sensory neuropathy is a rare syndrome characterized by the occurrence, in childhood or early adult life, of perforating ulcers of the feet, lightning pains, and loss of cutaneous sensation and tendon reflexes in the lower extremities. Three patients with hereditary sensory neuropathy were family members. Trophic ulcers may be caused by diseases other than diabetes, syphilis, and leprosy.

Adult↗
PubMed1978

Sialidase (alpha-n-acetyl neuraminidase) deficiency: the enzyme defect in an adult with macular cherry-red spots and myoclonus without dementia.

A 31-year-old male is described who has macular cherry-red spots, increased deep tendon reflexes and and myoclonus without dementia. An older brother died at age 33 of a disease with similar symptomatology. Homogenates of cultured fibroblasts from the patient exhibited 2.6, 8.1 and 12.4% of normal mean sialidase (neuraminidase, N-acetyl-neuraminosyl glycohydrolase, EC 3.21.18) activity, respectively, against 2-(3'-methoxyphenyl)-N-acetyl-alpha-neuraminic acid, N-acetyl-neuramin-lactose and fetuin. Activities of 14 other lysosomal enzymes were within the range of normal control fibroblasts. The sialidase activities in fibroblasts from the patient's parents and children were 30 to 67% of normal. It is concluded that this is the first proven case of a new autosomal recessive disorder resulting in cherry-red spots, myoclonus and a sialidase deficiency.

Adult↗
PubMed1993

Cervical epidural steroid injection for cervicobrachialgia.

Fifty patients with chronic resistant cervicobrachialgia were randomly divided into two groups. Twenty-five patients (group A) were treated with cervical epidural steroid/lidocaine injections and 17 patients (group B) were treated with steroid/lidocaine injections into the posterior neck muscles. Another eight patients from group B were excluded from the study because they had started the process of litigation of insurance claims and their subjective analysis of pain relief might therefore not be trustworthy. One to three injections were administered at 2-week intervals according to the clinical response. All patients continued their various pre-study treatments: non-steroidal anti-inflammatory drugs, non-opioid analgesics and physiotherapy. Pain relief was evaluated by the visual analogue scale 1 week after the last injection and then 1 year later. One week after the last injection we rated pain relief as very good and good in 76% of the patients in group A, as compared to 35.5% of the patients in group B. One year after the treatment 68% of the group A patients still had very good and good pain relief, whereas only 11.8% of group B patients reported this degree of pain relief. These differences were statistically significant. We failed to achieve significant improvement of tendon reflexes or of sensory loss in both groups, but the increase in the range of motion, the fraction of patients who were able to decrease their daily dose of analgesics, and recovery of the capacity for work were significantly better in group A. We encountered no complications in either group of patients.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗
PubMed1976

Hypokalemic myopathy due to chronic alcoholism.

A patient with hypokalemic myopathy occurring in the context of chronic alcoholism was reported. A 56-year-old male patient, heavy drinker for 20 years, complained of marked weakness and acutely developing pains in his limbs. The principal clinical findings were weakness and tenderness of the proximal limbs and girdle muscle. He was unable to lift his head or any extremities from the bed. Deep tendon reflexes were diminished, but not absent. There was no sensory disturbance except for muscle tenderness. These clinical manifestations disappeared gradually by abstinence from drinking, and potassium administration therapy, and the patient recovered completely on the 26th day after onset. On the day after admission (8th day), serum potassium value was 2.2 mEq/L, and serum CPK activity was 4270 IU. The ECG pattern was consistent with a diagnosis of low potassium content in serum, and the EMG pattern was consistent with a diagnosis of myopathy. These electrophysiological findings had a tendency to recover from this pattern to normal range correspondingly with clinical improvement. The repeated muscle biopsies showed that vacuolation, hyaline degeneration and significant phagocyte infiltration were observed in the muscle on the 9th day after the onset of muscle weakness, and that these pathological findings disappeared almost completely three weeks later. The frequently repeated examinations of potassium content and CPK activity in sera showed that there was a close correlation between these biochemical abnormalities and clinical improvement. The pathogenesis of alcoholic myopathy and significance of CPK abnormality in chronic alcoholism were discussed.

Adult↗