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[Subacute sensory neuropathy associated with carcinoma--an autopsy case report and an analysis of Japanese cases].

A 68-year-old man was admitted to our hospital because of numbness in the hands and feet, and unsteady gait in August, 1986. On neurological examination, deep tendon reflexes were absent in all limbs without pathological reflexes. Superficial and deep sensory disturbances of a glove and stocking type up to the level of the elbow and the knee were observed. Pseudoathetosis was noted in the hands. His gait was ataxic and Romberg sign was positive. Muscle strength was slightly decreased. Sural nerve biopsy showed severe loss of large myelinated fibers. Laboratory studies for malignancy showed lung cancer (Squamous cell carcinoma). Left pneumonectomy was performed in November, 1986, but he died in March, 1987. At autopsy, neither metastasis nor direct infiltration of malignant cells in the central and peripheral nervous systems were present macroscopically and histopathologically. Degeneration of the dorsal root ganglion and the posterior columns of the spinal cord were remarkable. Since 1955, only 9 cases of subacute sensory neuropathy had been reported in Japan. We analysed 10 Japanese cases (including our case) to clarify the clinicopathological features of subacute sensory neuropathy. Clinically, ataxic gait, paresthesia, deep sensory disturbance, and depression of deep tendon reflexes were present in the majority of the cases. Neuropathologically, neuronal cell loss and degeneration of the dorsal root ganglion, posterior roots and posterior columns of the spinal cord were universal findings.

Acute Disease↗

[HTLV-I associated myelopathy with Hashimoto's thyroiditis--a report of two cases].

We reported two cases of HTLV-I associated myelopathy with Hashimoto's thyroiditis. Case 1 is a 47-year-old female, who has had Hashimoto's thyroiditis since the age of 29. At the age of 38, she first noticed difficulty in walking which progressed gradually. At the time of examination, she showed a spastic gait, increased deep tendon reflexes in four extremities, hypesthesia of the feet, mild bowel and bladder disturbances. Anti-HTLV-I antibody was increased to the titer of 16,834 in the serum and of 32 in the cerebrospinal fluid by PA method. Hypothyroidism was present and the titers of microsome test and thyroid test were elevated to 102,400. Anti-T4, and anti-T3, and anti-thyroglobulin antibodies were found. Ultrasonography of the thyroid revealed its enlargement and decreased echo signals. Thyroid scintigram showed inhomogeneous 123I uptake and some defective areas of the uptake. Histological examination of the biopsy specimen of the thyroid demonstrated marked lymphocytic infiltration, germinal centers, hypertrophy of follicular epithelium, and interstitial fibrosis, which were consistent with Hashimoto's thyroiditis. Case 2 is a 60-year-old female who has had slowly progressive gait disturbance since her childhood. She became unable to walk at the age of 35. At the time of examination, she had a spastic gait, increased deep tendon reflexes bilaterally, and positive Babinski signs. Anti-HTLV-I antibodies were increased to a titer of 8,192 in the serum and of 32 in the cerebrospinal fluid. Thyroid function was normal. However, anti-thyroid antibodies were positive and histology of the thyroid showed lymphocytic thyroiditis, which were consistent with mild grade of Hashimoto's thyroiditis.(ABSTRACT TRUNCATED AT 250 WORDS)

Autoimmunity↗

Muscle responses and monosynaptic reflexes in falling monkey. Role of the vestibular system.

The free fall has been used in our laboratory as a way to test vestibular function in baboons in order to quantify vestibular compensation in the hemilabyrinthectomized animal. This study presents only those results that concern the contribution of the vestibular system to muscle responses due to sudden fall. EMG activity was recorded from the fully conscious animal using chronic electrodes implanted in various muscles. Spinal monosynaptic reflexes (Hoffmann's and tendon reflexes) were studied in the soleus muscle. Baboons were seated in a special chair suspended from an electromagnet and unexpectedly dropped 90 cm. Experiments were performed in normal, unilateral and bilateral vestibular neurectomized baboons. 1. In normal baboons, results showed a first short-latency response in all tested muscles, followed by a second peak of EMG activity in these muscles. Comparison with data from bilateral vestibular neurectomized baboons demonstrates that normal vestibular function is essential for the appearance of the first peak; the second peak rapidly disappears in our experimental situation where the animal's fall is mechanically braked and interrupted, so the animal does not have to make the postural adjustments necessary for landing, It is suggested that the first peak is concerned with the automatic and reflex control of landing, the second with the voluntary breaking of landing. 2. The modulation of monosynaptic spinal reflexes is closely related to the EMG response in soleus muscle. Facilitation of the H-reflex begins just prior to the onset of the EMG activity and continues as long as the baboon is falling. The T-reflex modulation presents a similar time course except in its early phase where it is depressed. Decrease in T and increase in H-reflexes suggest that the EMG response is most likely due to direct activation of alpha-motoneurons and not by means of the gamma-loop. 3. In unilateral vestibular neurectomized baboons, EMG and reflexological data show the classical asymmetry characterized by a strong decrease of the responses on the side of the lesion, and by a pronounced increase on the contralateral side. It is concluded that this represents the imbalance between the resting discharge of the vestibular neurons, and discloses the influence of labyrinthine afferences at the spinal level. We suggest consequently the use of EMG responses and modulation of spinal reflexes to fall in order to quantify vestibular compensation.

Animals↗

The prevalence and possible prognostic importance of arthropathy in childhood diabetes.

Specifically defined arthropathy was studied in 95 children with insulin-dependent diabetes mellitus. Thirty-eight patients (40%) had limited joint mobility (p = less than 0.001 vs healthy controls). Children with the following extraarticular findings were more likely to have arthropathy: tight skin of the hands (p = less than 0.001), retinopathy (p = less than 0.05), and/or decreased deep tendon reflexes (p = less than 0.01). When the frequency of articular findings was analyzed as a function of disease duration, a nearly linear increase in the percentage of children having joint findings was evident: 0-3 years, 28%, 4-8 years, 41%: greater than 8 years, 67%. The percentage of patients positive for any microvascular complication during these intervals rose much less sharply: 4-11%, 4-15%, and 24-52%. Since the fraction of children without any joint abnormality constantly decreases, the finding of arthropathy has little value beyond knowledge of disease duration as a predictor of the development of microvascular complications.

Adolescent↗

Reliability of the NINDS Myotatic Reflex Scale.

The assessment of deep tendon reflexes is useful for localization and diagnosis of neurologic disorders, but only a few studies have evaluated their reliability. We assessed the reliability of four neurologists, instructed in two different countries, in using the National Institute of Neurological Disorders and Stroke (NINDS) Myotatic Reflex Scale. To evaluate the role of training in using the scale, the neurologists randomly and blindly evaluated a total of 80 patients, 40 before and 40 after a training session. Inter- and intraobserver reliability were measured with kappa statistics. Our results showed substantial to near-perfect intraobserver reliability, and moderate-to-substantial interobserver reliability of the NINDS Myotatic Reflex Scale. The reproducibility was better for reflexes in the lower than in the upper extremities. Neither educational background nor the training session influenced the reliability of our results. The NINDS Myotatic Reflex Scale has sufficient reliability to be adopted as a universal scale.

Adult↗

The influence of age on patellar reflex response in the dog.

The patellar tendon reflex is a clinical parameter commonly used to assess neurological status. Factors such as quadriceps muscle fiber composition, femur length, and patient age have been reported to influence total and fractionated reflex times in human beings. The purpose of this blinded, cross-sectional study was to examine the effect of age on the patellar reflex in the dog. Eighty-six dogs without evidence of neurological impairment or orthopedic disease of the stifle (eg, patellar luxation or cranial cruciate ligament rupture) were assigned to 1 of 2 groups on the basis of age: group 1, <10 years old (n = 72); or group 2, > or =10 years old (n = 14). Patellar reflexes were elicited in both pelvic limbs by a reflex hammer while the dog was in lateral recumbency. The reflex was scored as present or absent by a blinded individual. Two dogs in group I had reflexes absent in both limbs, 3 dogs in group 2 had reflexes absent in both limbs, and 1 dog in group 2 lacked a response in 1 limb. The proportion of dogs with absent patellar reflexes in group 2 (4 of 14, 95% CI, 8-58%) was significantly higher than the proportion of dogs with absent patellar reflexes in group 1 (2 of 72, 95% CI, 0-10%) (P < .006). Furthermore, dogs lacking at least 1 patellar reflex were older than those having both reflexes present (P = .04). Weight was not related to the presence of both reflexes (P = .49). These findings suggest that neurologically normal dogs may have an age-dependent decline in patellar reflex magnitude or a prolongation of total reflex time (TRT).

Aging↗

Prevalence of upper motor neuron vs lower motor neuron lesions in complete lower thoracic and lumbar spinal cord injuries.

OBJECTIVE: To determine the incidence and etiology of lower motor neuron (LMN) vs upper motor neuron (UMN) lesions in patients with complete thoracic and lumbar spinal cord injuries (SCI). DESIGN: Retrospective chart review. SETTING: A regional Model Spinal Cord Injury System center. METHODS: A consecutive sample of medical records of patients with lower thoracic and upper lumbar (T7-L3) complete SCI admitted from 1979 through 1996 was systematically reviewed. Of the 306 patients evaluated, 156 subjects met inclusion criteria. The incidence and etiology of LMN vs UMN lesions were determined for the following neurologic levels: T7-T9, T10-T12, L1-L3. Lesions were classified as LMN, UMN, or mixed on the basis of the presence or absence of (1) the bulbocavernosus reflex, (2) lower limb deep tendon reflexes below the neurologic level of injury, and (3) the Babinski sign. RESULTS: The incidences of LMN, UMN, and mixed lesions in the T7-T9, T10-T12, and L1-L3 groups were as follows: T7-T9 group (7.3% LMN, 85.5% UMN, 7.3% mixed), T10-T12 group (57% LMN, 17.7% UMN, 25.3% mixed),L1-L3 group (95.5% LMN, 0.0% UMN, 4.5% mixed). Etiology of injury did not significantly influence the likelihood of a LMN lesion. CONCLUSIONS: One cannot determine the type of lesion (UMN vs LMN) on the basis of the neurological level of injury. A detailed clinical examination, including sacral reflexes, is required. This has important prognostic and therapeutic implications for bowel, bladder, and sexual function, as well as mobility. Distinguishing UMN lesions from LMN lesions is also essential for evaluating new interventions in clinical trials for UMN pathology.

Cohort Studies↗

The relation between monosynaptic spinal reflex amplitudes and some EEG alpha activity parameters.

Some investigators have found that in normal human subjects the amplitudes of repetitively evoked tendon reflexes decrease as the number of evoked reflexes progresses. The question whether this decrement is a spinal phenomenon or that it can be ascribed to supraspinal influences that are related to the degree of cortical activation was investigated in two experiments designed to test whether a relation exists between the Hoffmann (H) and Achilles tendon (T) reflex amplitudes and EEG alpha activity parameters during a rest condition. The principal results can be summarized as follows: 1. A constant alpha index was accompanied by stable reflex amplitudes. 2. A decreasing alpha index was accompanied by decreasing reflex amplitudes. 3. A positive relation was found between H and T reflex amplitudes. 4. A positive relation was found between alpha index and alpha amplitude. 5. No circumscribed relation was found between the alpha wave period on the one side and the alpha index and amplitude on the other side. Apparently these relations are dependent on the state of activation of the subject. 6. The relations between the reflex amplitudes and the alpha parameters showed great interindividual differences.

Adult↗

[Examination of the unconscious patient].

The unconscious patient presents an challenging emergency in the intensive care unit. Patient evaluation and treatment must occur simultaneously. After checking vital signs, deciding about cardiopulmonary resuscitation or intubation, an intravenous line must be established, blood samples collected and glucose and thiamine should be given. The physical and neurological examination should be performed in a efficient way and must consider the following eight points: best reaction to loud commands, best motor reaction to noxious stimuli, respiratory pattern, size and reactions of pupils, eye movements, corneal and blink reflex, meningismus, muscle tone and deep tendon reflexes. With this approach it is possible to formulate a working diagnosis, and the appropriate diagnostic steps can be performed.

Coma↗

Anti-GQ1b-negative Miller-Fisher syndrome with lower cranial nerve involvement from parasinusoidal aspergilloma.

Miller-Fisher syndrome (MFS) typically presents with ophthalmoplegia, ataxia, and areflexia. Atypical MFS additionally includes bulbar impairment, affection of the limbs, or abortive presentations. Mostly, MFS follows an infection with Campylobacter jejunii. Aspergilloma has not been reported to trigger MFS. In a 48-year-old male tiredness, tinnitus, otalgia, parietal hyperaesthesia, coughing, plugged nose, hypoacusis, globus sensation, epipharyngeal pain, dysarthria, hypogeusia, arthralgia, lid cloni, facial hypaesthesia and tooth ache consecutively developed. There were occasional lid cloni, left-sided facial hypaesthesia, reduced gag reflex, divesting soft palate, and absent tendon reflexes. CSF investigations revealed normal cell-count but increased protein. Antibodies against GM1 and GQ1b were negative. Atypical MFS was diagnosed. Otolaryngological examinations revealed chronic sinusitis maxillaris from an aspergilloma. After immunoglobulins and resectioning of the aspergilloma, neurological abnormalities disappeared within 19d. MFS may manifest as unilateral lower cranial nerve lesions without affection of the upper cranial nerves or ataxia. Atypical MFS may be triggered by parasinusoidal aspergilloma.

Aspergillosis↗

Atypical spinal dural arteriovenous fistula with supply from the lateral sacral artery.

We report a dural arteriovenous fistula (AVF) that developed at a site on the midline dorsal surface of the dura mater that had been damaged by repeated lumbar punctures. A 61-year-old male patient had undergone repeated lumbar punctures and discectomy for severe lumbago 40 years before the present admission. After surgery, the lumbago symptoms resolved. However, 30 years after the operation, he started to experience dysaesthesia, motor weakness in both legs, and urinary disturbance. Physical examination revealed bilateral leg weakness, diminished deep tendon reflexes in the patellar and Achilles tendons bilaterally, and decreased superficial sensation below L1. Magnetic resonance imaging revealed swelling with intramedullary high intensity and multiple flow voids around the conus and spinal cord on T(2)-weighted images, and adhesive arachnoiditis. Spinal angiography revealed an AVF between the left lateral sacral artery and the S1 radicular vein at the site of the previous operation. Surgery was conducted to carry out excision of the dural AVF at the shunting point, the arterialized intradural vein, and lysis of the arachnoiditis. This case of dural AVF may have been caused by repeated lumbar punctures.

Arteriovenous Fistula↗

[Prolonged neuromuscular damage following corticosteroids and muscle relaxants].

Many patients mechanically ventilated for acute respiratory failure, are treated with medication that includes a combination of cortico-steroids and non-depolarizing neuromuscular-blocking agents (NNBa). A third of them can be expected to develop delayed neuromuscular damage, which may be severe and prolonged. We describe a 50-year-old man who suffered from acute myeloid leukemia and was ventilated due to pneumonia. He was treated with pancuronium and cortico-steroids, and during recovery suffered quadriparesis that lasted several months. Typically this damage is purely motor and is accompanied by absent tendon-reflexes, sometimes with elevated creatine-kinase. Muscle biopsy usually shows deletion and degeneration of thick myosin filaments. The phenomenon is related to the duration of NNBa treatment, and probably results from an adverse synergistic effect on muscle tissue of the cortico-steroids and cortico-steroid-like NNBa given the immobilized patient. Awareness of this adverse effect of steroids and pancuronium, the use of passive mobilization, shortening the use of NNBa and early rehabilitation would minimize disability due to this phenomenon.

Adrenal Cortex Hormones↗

Nitrous oxide depresses the H-reflex in children with cerebral palsy.

Hoffmann's reflex or H-reflex (HR) is an electrically elicited reflex that measures excitability of motoneurons and shares some physiologic properties with the deep tendon reflex. Children with tendon hyperreflexia due to cerebral palsy usually have higher amplitude HRs. Nitrous oxide (N2O) depresses the HR in patients with normal spinal reflexes, although the effect of N2O in conditions with hyperreflexia such as cerebral palsy is not known. We propose to determine the effect of N2O on the amplitude of the HR under general anesthesia in children with hyperreflexia due to cerebral palsy. We studied eight children undergoing selective dorsal rhizotomy (SDR) for the relief of spasticity. The maximum amplitudes of the HR (HRmax) and direct motor response (MRmax) were routinely evoked under the following anesthetic conditions: 1) sufentanil and 66% N2O/33% oxygen; and 2) sufentanil and 100% oxygen. The HRmax amplitude was significantly lower when N2O was part of the inspired gas mixture. The differences between the no N2O and the 66% N2O groups were significant. The MRmax did not change significantly. Abnormal spinal reflexes seen in spastic diplegia can be abolished by inhaled N2O. This finding also suggests that N2O-induced depression of spinal reflexes should be a consideration during physiologic monitoring of the spinal cord under general anesthesia.

Cerebral Palsy↗

Mechanically induced reflex responses in human triceps brachii.

The short and long latency reflex responses of human triceps brachii muscle were recorded in 14 healthy volunteers. An electromechanical hammer was used to stretch the muscle and recordings were made from a surface electromyogram. The monosynaptic tendon reflex occurred at a mean latency of 12.5 ms (SE 0.7 ms). Later responses were observed in activated conditions (weak force production, preparatory period) at a mean latency of 62.8 ms (SE 3.5 ms). The amplitude of the short latency reflex increased during weak tension, the long latency reflex amplitude seemed to increase during the preparatory period testing. The amplitude increases can be attributed to increased lower motoneuron excitability even during weak voluntary activity. The tendency towards an increased amplitude during the preparatory period may be connected with the higher regulation of the long latency reflex.

Adolescent↗

[Focus on current research: improving the mobility of paraplegic patients].

Since the first paraplegic centre was established in 1945, life expectancy and life quality of paraplegics have considerably improved. However, endeavours to enhance the mobility of these patients have been less successful. The most promising approach, functional electric stimulation of paralysed muscles, is poorly accepted by patients at present because of technical problems. This study describes current approaches which may help to improve patients' mobility. A central motor lesion is perceived by the patient as a movement disorder of the legs, e.g. a gait disorder. Neurological investigation indicates, on the basis of exaggerated tendon reflexes and increased resistance of the non-activated leg muscles to stretching, that spastic paresis underlies the movement disorder. This combination of symptoms and clinical (physical) signs suggests that the exaggerated tendon tap reflexes are responsible for muscle hypertonia and the latter causes the movement disorder. However, electromyography during movement shows that the exaggerated short latency reflexes are associated with loss or attenuation of the functionally essential polysynaptic spinal reflexes. In the event of impaired supraspinal control there is loss of monosynaptic stretch reflex inhibition combined with reduced facilitation of polysynaptic spinal reflexes. Development of tension in tonically active calf muscles in patients with spastic paresis during gait occurs independently of spinal reflex activity. From electrophysiological and histological observations it can be assumed that transformation of motor units resulting in simple and less well adapted regulation of muscle tone allows movements such as gait. The reduction of muscle tone obtained with antispastic drugs is usually associated with paresis and may therefore hamper locomotion. Locomotor training represents a new attempt to improve the mobility of patients with incomplete paraplegia. It includes activation of neuronal circuits within the spinal cord below the level of the lesion. In incomplete paraplegics a coordinated leg muscle activation pattern and corresponding leg movements can be triggered and trained in patients standing on a treadmill with partial weight support. Improvement of training of the spinal cord locomotor centre can be expected from triggering of spinal cord reflexes and regeneration of spinal tract fibres, which is expected to be possible in the near future.

Humans↗

Changes with age in monosynaptic reflexes elicited by mechanical and electrical stimulation.

Absence of the achilles tendon reflex (T) has been reported to increase with age. Other investigators have reported no age relationship. Age changes in the Hoffmann reflex (H) have been examined in only one recent investigation. It was our purpose to compare the T and H reflexes of healthy, active old (n = 20) against young (n = 20) subjects using methods which provide stimulus-response data for both T and H. We also compared the age differences of H against T to estimate fusimotor involvement in age changes. No significant age difference were found in the response:stimulus ratio for T. Amplitude of H responses were 32.7 percent smaller in the old (P less than 0.04) and M waves were smaller by 24 percent (P less than 0.025). No significant age difference was found between mechanically and electrically elicited reflexes; and, therefore, our data do not support fusimotor involvement in the age changes of monosynaptic reflexes.

Achilles Tendon↗

Mechanisms of tetrazepam action on spasticity.

This investigation assessed the mechanisms of Tetrazepam action on spasticity using a battery of electromyographic methods. Thirty patients with post-stroke spastic hemiparesis treated with Tetrazepam took part in the investigation. A questionnaire for assessment of subjective improvement after treatment used a 5-point scale. The 5-point scales were used to assess muscle tone, muscle strength and tendon reflexes. A battery of electromyographic methods was used to analyse different mechanisms of spasticity: for alpha-motoneuron activity--the F-wave parameters; for gamma-motoneuron activity--the TA/H amplitude ratio; for presynaptic inhibition--the ratio of H-reflex maximal amplitudes before and after vibration on the Achilles tendon (Hvibr/Hmax); for common interneuron activity--the flexor reflex parameters. Our results revealed that Tetrazepam reduces tone in spastic muscles and has a slight effect on tendon hyperreflexia. It has no influence on muscle strength, Babinski sign and ankle clonus. Tetrazepam acts by decreasing motoneurone activity and increasing presynaptic inhibition.

Aged↗