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Excitability of lower limb myotatic reflex arcs under the influence of caloric labyrinthine stimulation. Analysis of the postural effects in man.

The excitability changes of myotatic reflex arcs have been investigated in 36 volunteers by two methods (tendon reflex and tonic vibration reflex) in a bilateral invertigation of soleus, quadriceps, biceps femoris, and tibialis anterior after caloric stimulation of the labyrinth. The extensor myotatic reflexes are facilated during the irrigation and the nystagmus phases. Contrary to the soleus, quadriceps facilitation is not symmetrical during the phase of nystagmus but predominates on the side opposite to the expected axial deviation. The short biceps femoris reflexes are slightly facilitated. The postural modifications arise from differences in the degree of bilateral facilitation of the extensors.

Adolescent↗

Jaw reflex in Friedreich ataxia.

Twenty-one patients with Friedreich ataxia were examined and found to have a brisk jaw reflex. This previously unreported clinical finding contrasts with the impairment of tendon reflexes in the limbs and should not rule out the diagnosis of Friedreich ataxia.

Adolescent↗

Electromyographic recording of the jaw reflex in Friedreich ataxia.

Jaw jerk was studied in 10 subjects with Friedreich ataxia, and 10 normal subjects for comparison. Electromyographic recording from the right masseter muscle showed a reflex response in all cases, in contrast to the absence of tendon reflexes in the four limbs for the ataxics. The mean latency of the jaw jerk was normal. These observations indicate that the jaw reflex arc is preserved, and that no damage to the trigeminal neuromuscular pathway exists for all the patients observed.

Adult↗

H-reflex: optimum location of recording electrodes.

The Hoffmann (H) reflex was measured from a grid of 31 locations on the calves of 20 healthy volunteers. In 17 cases the maximum amplitude of response was found at a site more distal than at the conventional midcalf recording location. Three quarters of the distance between the popliteal crease and the flare of the medial malleolus, the waveform was found most consistently triphasic. At this site the latency was, on the average, 1.4msec longer than at the midcalf location. These results may be related to the more distal origin of the H-reflex dipole when compared to the M (motor) wave or T (tendon) reflex response, and indicate that a distal recording site is preferred if the H-reflex per se is to be assessed.

Adult↗

Extensor digitorum brevis reflex in normals and patients with radiculopathies.

This prospective study evaluated the extensor digitorum brevis deep tendon reflex (EDBR) in a normal population and in patients with L-5 and S-1 radiculopathies. There were 88 subjects: 53 normals, 17 L-5, and 18 S-1 radiculopathy subjects. The clinical EDBR revealed a 91% specificity, with 18% sensitivity for L-5, and 11% for S-1. The electrodiagnostic EDBR yielded increased sensitivities of 35% for L-5 (P = 0.07) and 39% for S-1 (P = 0.04), with 87% specificity. H-reflexes showed 50% sensitivity for the S-1 group (P = 0.0006) and 91% specificity. EDBR latencies were significantly related to age and leg length (r2 = 0.46, P < 0.0001). Age alone explained 26% (P < 0.0001) of the EDBR variability and leg length 20% (P < 0.0001). EDBR clinical and electrodiagnostic reflexes have low sensitivities, high specificities, and do not discriminate L-5 from S-1 root involvement.

Adult↗

Quantitative evaluation of reflex and voluntary activity in children with spasticity.

OBJECTIVE: To determine whether increased reflexes are related to functional impairment in children with spasticity. DESIGN: Descriptive measurement study. SETTING: Rehabilitation department in Poland. PARTICIPANTS: Sixteen able-bodied children and 29 children with spasticity. INTERVENTIONS: Not applicable. MAIN OUTCOME MEASURES: Measurement of modulation function of knee tendon reflexes by isometric knee extension, maximum isometric knee flexion, and extension torques, and scoring of ambulation in patients. RESULTS: In able-bodied children, the reflex modulation function increased with voluntary knee extension, reached maximum at 5% to 20% of voluntary extension, and then decreased. The reflex modulation function in patients fell into 2 major categories. In the majority of affected limbs, the modulation function was inverse, with maximum reflex response at relaxation, and decreased with an increase of voluntary extension. In the remaining limbs, the shape of the reflex modulation function was normal, although other parameters changed. Isometric torques decreased more in flexion (65%) than in extension (39%). A torque decrease was a result of cocontraction more often during knee flexion (65%) than in knee extension (24%). The larger the reflexes, the more flexion torque decreased and ambulation deteriorated. This pattern occurred in patients with inverse modulation function but not in those with normal modulation function. The reciprocal inhibition from knee flexors to extensors could be affected in patients with inverse modulation function and cocontraction during flexion, whereas other mechanisms occurred in other patients. CONCLUSIONS: The experimental design has potential as a quantitative measure of abnormal control in children with spasticity and can lead to more precise treatment selection criteria.

Adolescent↗

Amelioration of vincristine neurotoxicity by glutamic acid.

Neurotoxicity is the principal limiting side effect of the widely used antitumor agent, vincristine. Following evaluation of glutamic acid as a potential modifier of vincristine toxicity in preclinical studies in mice and a preliminary clinical trial, a prospective, double-blind, placebo-controlled, randomized trial was conducted by the Piedmont Oncology Association. Of 87 patients entered into the study, 84 were evaluable, including 42 patients who were randomly assigned to receive vincristine 1.0 mg/m2 weekly for six doses and 42 patients who were assigned to receive glutamic acid 500 mg orally three times daily plus vincristine. The following neurotoxic signs and symptoms were evaluated before each dose of vincristine: reflex changes, paresthesias, constipation, strength, and mental changes. Loss of the Achilles tendon reflex, an objective parameter, was noted in 19 percent of patients receiving glutamic acid and 42 percent of control subjects (p = 0.03). Development of moderate to severe paresthesias, a subjective parameter, occurred in 19 percent of the glutamic acid group and 36 percent of the placebo group (p = 0.09). Overall moderate neurotoxicity (6 units or more), determined by adding the grade of each neurotoxic parameter for the weekly clinic visit in which maximum neurotoxicity occurred, was observed in 21 percent of patients receiving glutamic acid and 43 percent of those in the control group (p = 0.04). Hematologic and gastrointestinal side effects occurred with similar frequency in the two groups. The administration of glutamic acid has decreased vincristine-induced neurotoxicity without any attendant side effects.

Adult↗

A controlled trial of sorbinil, an aldose reductase inhibitor, in chronic painful diabetic neuropathy.

A double-blind, randomized, placebo-controlled cross-over trial of the aldose reductase inhibitor sorbinil was undertaken in 15 patients (age 35-68 yr) with chronic painful diabetic neuropathy. Treatment was evaluated by subjective pain responses, clinical examination, vibration perception threshold, motor and sensory nerve electrophysiology, and cardiovascular reflex tests of autonomic nerve function. Among the many measurements, only pain, tendon reflex scores, and sural sensory potential amplitude improved significantly during sorbinil administration, while scores of clinical sensory examination deteriorated. Four patients experienced an idiosyncratic reaction that rapidly recovered on discontinuing the drug. This study suggests that aldose reductase inhibitor treatment with suggests that aldose reductase inhibitor treatment with sorbinil may have an effect on symptomatic diabetic neuropathy in man.

Adult↗

Scorpionism in South Africa. A report of 42 serious scorpion envenomations.

Forty-two cases of serious scorpion envenomation, of which 4 had a fatal outcome, are presented. The clinical profile, differential diagnosis and management of scorpionism are discussed. Most envenomations occurred in the summer months, peaking in January and February. An immediate local burning pain was the most prominent symptom. Systemic symptoms and signs developed within 4 hours of the sting in most instances, characterised by general paraesthesia, hyperaesthesia, muscle pain and cramps. Other striking features included dysphagia, dysarthria and sialorrhoea with varying degrees of loss of pharyngeal reflexes. The blood pressure and the temperature were often raised and the tendon reflexes increased, while motor power was often impaired. In a considerable number of patients the course was complicated by varying degrees of respiratory dysfunction, which tended to be more serious in children. The oustanding feature in children was an extreme form of restlessness characterised by excessive neuromuscular activity. Victims of scorpion sting, particularly in high-risk localities, should be closely observed for 12-24 hours. Children and other high-risk patients should be hospitalised. All patients with symptoms and signs of systemic envenomation should receive antivenom. Parabuthus granulatus (Hemprich & Ehrenberg, 1828) has been identified as the most important venomous species in the western Cape. The antivenom is produced from the venom of the medically less important P. transvaalicus Purcell, 1899. A strong case can therefore be made for the inclusion of P. granulatus venom in the production of a polyvalent antivenom.

Adult↗

Motor compensatory reactions following a forward fall in subjects with unilateral abolition of the triceps-surae H reflex.

The electromyograms of the right and left soleus and tibialis anterior muscles of 6 subjects with unilateral abolition of Achilles tendon reflex due to S1 radiculitis were recorded during a forward fall involving stepping to recover balance. Each subject took part in two series of experiments, one in which the step was performed with the unaffected leg and a second in which the affected leg was used. A unilateral deficiency of peripheral proprioceptive afferents affected ankle muscles EMG activities bilaterally, except for the EMG activity of the soleus of the starting foot. The tibialis anterior of both the affected and the unaffected side, showed either a normal pattern (i.e. a phasic contraction after soleus contraction stopped), or an early contraction. On the stance side, the early contraction was associated with a depressed soleus EMG activity. Some abnormal motor patterns could be due to the ipsilateral deficiency of the Ia inhibitory projection from soleus to tibialis anterior. The presence of abnormal patterns on the unaffected side indicates that the motor activity in one lower limb can be modified by a loss of peripheral afference in the contralateral limb. This suggests that crossed pathways between lower limbs are involved in balance recovery movement.

Achilles Tendon↗

Reduced Ia-afferent-mediated Hoffman reflex in streptozotocin-induced diabetic rats.

In addition to reduced nerve conduction velocity, diabetic neuropathic patients often exhibit a reduction in the amplitude of the compound muscle action potential elicited by stimulation of the Ia-afferent-mediated reflex pathway (Hoffman or H wave) that can contribute to diminished or absent tendon reflexes. In contrast to nerve conduction velocity deficits, changes in H-wave amplitudes have not been reproduced in diabetic animal models. Using electrophysiological techniques developed for repeated recordings in individual animals, we report H-wave deficits in streptozotocin (STZ)-treated insulin-dependent diabetic rats. After 4 weeks of diabetes induced by STZ treatment, a 47% reduction in the H-wave amplitude was demonstrated by recording compound muscle action potentials in foot muscles after stimulation of Ia afferents. Interestingly, we also demonstrate that the H-wave amplitude gradually recovers to a 26% deficit after 12 weeks of experimental diabetes. The recovery of the H wave in STZ-treated rats distinguishes this deficit mechanistically from other STZ-induced electrophysiological changes and may model a similar recovery of the H wave reported in diabetic patients.

Action Potentials↗

Studies on the aetiology and pathogenesis of motor neuron diseases. 1. Lathyrism: clinical findings in established cases.

Lathyrism is a toxic disease of the motor system constantly associated with primary consumption of the seed of Lathyrus sativus (chickling or grass pea). Neurological examination was performed on 38 affected subjects drawn from four regions of the Indian subcontinent endemic for lathyrism. All showed a consistent and largely symmetric pattern of neurological deficit similar to that displayed by 5 Europeans with longstanding lathyrism now living in Israel. Hallmarks of lathyrism include a pyramidal pattern of motor weakness combined with greatly increased tone in the thigh extensors and adductors and in the gastrocnemius muscles so that the more severely affected walk on the balls of their feet with a lurching scissoring gait. Extensor plantar responses are uniformly present in such cases, and the knee and ankle tendon reflexes are exaggerated and often clonic. Hoffmann signs and exaggerated biceps and/or triceps tendon jerks are also found in the most severely affected. Sensory signs are absent, although perverse sensations in the legs are frequently reported at the onset. Walking difficulties commonly begin suddenly but may also appear subacutely or insidiously. Some individuals experience partly reversible symptoms suggestive of a diffuse CNS excitation of somatic, motor and autonomic function.

Adult↗

[Internal ophthalmoplegia in acute oropharyngeal palsy with anti-GQ1b and anti-GT1a IgG antibodies].

A patient with acute oropharyngeal palsy associated with internal ophthalmoplegia was reported. A 13-year-old boy had fever and diarrhea for two days. Ten days after resolution of these symptoms, he noticed difficulty in speaking (day 1). Neurological findings on day 4 included bilateral mydriasis, right abducens nerve palsy, nasal voice with absent pharyngeal reflex. Although superficial sensation was preserved, vibratory sensation was reduced in distal limbs. Tendon reflexes were generally absent. Neither ataxia nor dysautonomia was seen. Serum anti-glycolipid antibody assay on day 4 disclosed elevated IgG antibodies to GQ1b and GT1a. His cerebrospinal fluid on day 21 contained 6 mononuclear cells/microl with 137 mg/dl of total protein. Nerve conduction study on day 5 showed minimal sensory nerve involvement. Quantitative sudomotor axon reflex test was normal in the lower extremities. Low dose pilocarpine eyedrops dilated his pupils. Although mild cerebellar-like ataxia appeared on day 5, intravenous immunoglobulin (0.4 g/kg/day for four days) rapidly improved his neurological abnormalities. IgG anti-GQ1b antibody might contribute not only oropharyngeal weakness but also internal ophthalmoplegia in this patient.

Acute Disease↗

[A case of adult T cell leukemia/lymphoma with motor and sensory polyneuropathy].

A 62-year-old man was admitted to our hospital because of two months continuing paresthesia and muscle weakness of distal portions of the four limbs. On general physical examination, skin lesions, lymphadenopathy and hepatosplenomegaly were not found. Neurological examination revealed moderate weakness in the bilateral distal muscles of the lower limbs and left distal muscles of the upper limbs, and slight weakness in the right distal muscles of the upper limbs and the bilateral proximal muscles of the four limbs. Hand grasping powers were 24 kg and 2 kg on the right and left, respectively. The biceps, triceps and radial reflexes were decreased on the right, but normal on the left. The Achilles tendon reflex was decreased on the right and absent on the left. Paresthesia and superficial sensory disturbance were observed with glove and stocking distribution, which was more severe on the left side. The vibration and position senses were slightly decreased in the distal part of the lower limbs. On the laboratory examinations, serum anti-HTLV-I antibody was positive and no abnormal lymphocytes were observed in peripheral blood. Cerebrospinal fluid findings were normal, and anti-HTLV-I antibody was negative. Motor and sensory conduction velocities were normal or slightly decreased in all of the limb nerves examined, but the amplitudes of the compound muscle action potentials and the sensory nerve action potentials were asymmetrically decreased. Needle EMG showed fibrillation potentials and giant spikes with a reduction in number of motor unit potentials. The histological examination of the biopsied sural nerve revealed severe axonal degeneration without evidence of vasculitis or infiltration of abnormal lymphocytes.(ABSTRACT TRUNCATED AT 250 WORDS)

CD4-CD8 Ratio↗

[A case of Down's syndrome with chronic inflammatory demyelinating polyradiculoneuropathy].

A 19-year-old male patient with Down's syndrome accompanied by relapsing and steroid-dependent chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) was reported. He had been well until the beginning of June 1988, and he rapidly developed a gait disturbance and symmetrical numbness in his arms and legs at the end of that month. He also suffered from difficulty in swallowing and dyspnea soon afterward. A physical examination revealed the specific clinical features of Down's syndrome, but no particular signs. Neurological examination showed the following abnormal findings; fixed nystagmus, diminished gag reflex, flaccid tetraparesis, and distal dominant dysesthesia of upper and lower extremities. Deep tendon reflexes were diminished and Babinski's sign was negative. No autonomic dysfunction was detected. Routine hematology and biochemistry screening test revealed normal level except for increasing GPT and IgG. Protein and gamma globulin values in cerebrospinal fluid (CSF) were elevated without pleocytosis. Nerve conduction velocities were slighty reduced. Pulse therapy and corticosteroid treatment improved on clinical course and CSF results. CIDP probably results from an abnormal immune responsiveness. Down's syndrome has often been taken for autoimmune abnormality. It is of great interest whether there is a close connection of autoimmune abnormality between Down's syndrome and CIDP. This might be the first case report of Down's syndrome associated with CIDP, judging from our survey of the literature in Japan and other countries.

Adult↗

[Recurrent episodic unilateral mydriasis with pleocytosis in the cerebrospinal fluid--a case report].

A 24-year-old female was admitted to our hospital on Aug. 20 in 1986 because of blurred vision and right pupillary dilatation. She had sometimes noticed headache later than 1976, and blurred vision without headache several times a year later than 1983. She had been told her right pupil dilated when she had complained of blurred vision. Neurological examination revealed abnormal findings as follows; diminished sense of smell in the right side, anisocoria (R 8 mm, L 5 mm), bilateral hippus, hypesthesioalgesia in her right face, left trunk and left arm. The pupils were round and contracted promptly to light. Accommodation reflex and ciliospinal reflexes were normal. Neither blepharoptosis nor external ocular muscle paresis were observed. Deep tendon reflexes were normal. Planter responses were flexor. There was no meningeal irritative sign. No abnormal findings were obtained in blood and urine, chest X-p, brain enhanced CT scan, EEG, and cerebral angiography except for slight degree of anemia. Serum TPHA was negative. However, the cell count of cerebrospinal fluid (CSF) was 18/mm3 (Ly 100%) and decreased to 9/mm3 (Ly 100%) in nine days. Protein content and glucose level of CSF were normal. Pupils were not constricted by 0.125% pilocarpine instillation. Loss of smell and sensory disturbance disappeared within three days and her pupils became isocoric by five days after admission. The patients of episodic unilateral mydriasis without apparent cause had relatively same clinical features as "unilateral springing pupil" proposed by Hallett et al. (1970). Except for mydriasis, they had no abnormal findings of neurological and laboratory examinations.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗

Interest of peripheral anesthetic blocks as a diagnosis and prognosis tool in patients with spastic equinus foot: a clinical and electrophysiological study of the effects of block of nerve branches to the triceps surae muscle.

OBJECTIVE: To evaluate clinically and electrophysiologically the effects of selective anesthetic blocks of motor nerve branches to the triceps surae muscle on lower limb stretch reflex in patients with spastic equinus foot. METHODS: Eleven patients were assessed before and after selective anesthetic block of the superior soleus nerve or the gastrocnemius nerves, performed by lidocaine injection. The stretch reflex (SR) of the ankle with the knee flexed or extended and the Achilles tendon reflex (TR) were scored clinically. Additionally, the direct M response and the H reflex to tibial nerve stimulation were recorded on the three heads of the triceps surae muscle. The ratio of H reflex to M response of maximal amplitudes (H(max)/M(max)) was calculated. RESULTS: The SR and TR mean scores were significantly reduced after soleus nerve block but not after gastrocnemius nerve block. Electrophysiologically, H(max) and H(max)/M(max) ratios were significantly reduced for the soleus muscle after soleus nerve block and for the lateral (but not medial) gastrocnemius muscle after gastrocnemius nerve block. CONCLUSIONS: Soleus nerve block appeared more appropriate than gastrocnemius nerve block to relieve spasticity clinically. In addition, the decrease in H(max)/M(max) ratio suggested that lidocaine preferentially blocked proprioceptive Ia fibers rather than A-alpha motor fibers. SIGNIFICANCE: Selective anesthetic blocks of nerve branches to the triceps surae muscle are useful in the assessment of lower limb spasticity and can benefit from H reflex investigation. H reflex recordings showed a preferential susceptibility of muscle spindle afferents to local anesthetics and supported the hypothesis of a prominent role of the soleus muscle in spastic ankle. The clinical and electrophysiological effects induced by anesthetic blocks may help to guide therapeutic interventions, such as neurotomy, neurolysis or botulinum toxin injection.

Adolescent↗