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Recurrent laryngeal nerve histopathology in spastic dysphonia: a preliminary study.

Spastic dysphonia is a severe voice disorder ordinarily described as psychogenic. Organic-neurologic changes secondary to central or peripheral nervous system involvement have also been postulated and led recently to the surgical treatment of spastic dysphonia by unilateral section of the recurrent laryngeal nerve (RLN). This study reports the histologic findings from these resections of the RLN from patients with an average 9.5 years duration of spastic dysphonia. Thirty percent of the RLNs examined demonstrated significant abnormalities as compared to age-matched controls. Although no reactive changes were noted by light microscopy, groups of fibers which did not stain for myelin or axons were found in RLNs from patients with spastic dysphonia. A preliminary ultrastructural study of these areas in one RLN revealed sheets of unmyelinated axons. These findings suggest an organic basis for spastic dysphonia at least in some patients.

Adult↗

Symptom improvement of spastic dysphonia in response to phonatory tasks.

It has been acknowledged that for many patients with spastic dysphonia, reflexive phonation remains essentially free of the spasticity that characterizes this disorder. The purpose of this paper is to document the extent to which various phonatory tasks change the patient's voice. We retrospectively reviewed 37 patients with spastic dysphonia evaluated at the Center for Communication Disorders of Lenox Hill Hospital, New York, between 1977 and 1981. The patients' responses to various phonatory tasks were observed. These tasks were grouped into the following ten categories: noncommunicative vocalization, primitive communication, speech superimposed on noncommunicative phonation, communicative phonation with varied mode of vocal fold vibration, normal communicative phonation with unusual pitch, normal communicative phonation with unusual emphasis, normal communicative phonation with normal laryngeal adjustments, use of the vocal folds in an artistic manner, speech in which normal auditory feedback was eliminated, and speaking with whisper which was not associated with vocal fold vibration. The results are summarized as follows: 1) whispered speech always resulted in an improvement of the symptom, in most cases markedly; 2) there was a tendency for a task that was more effective in reducing spasticity to be reduced in communicative function; 3) there was a tendency for a task that was more effective in reducing the spasticity to deviate more from the normal mode of phonation; and 4) there were some patients in whom an improvement occurred with elimination of auditory feedback.

Adult↗

Electromyographic findings in focal laryngeal dystonia (spastic dysphonia).

Spastic dysphonia is a clinical speech disorder characterized by spasms of the laryngeal muscles during phonation, producing a broken pattern of speech sometimes termed laryngeal stuttering. Fourteen patients with the diagnosis of spastic dysphonia based on voice quality were referred for evaluation; detailed clinical and electrophysiologic evaluations were performed. Laryngeal electromyographic (EMG) testing failed to demonstrate any spontaneous activity in the 14 patients tested. Seven patients (50%) had normal number and amplitude of motor unit potentials. Four of these had disparate amplitudes when compared with the other side, and two had complex motor unit potentials. The other seven patients (50%) had abnormal findings, including three patients with abnormally increased amplitude. Two patients had asynchronous activity characteristic of a tremor disorder. One patient had synchronous bursts of activity also affecting the diaphragm, later diagnosed as pyramidal and extrapyramidal disease. One patient had bursts of activity, and later presented with diffuse myoclonus. Laryngeal EMG therefore seemed to be a more precise way of evaluating patients presenting with a tremulous voice pattern termed spastic dysphonia. Clinical observation and EMG data demonstrated that spastic dysphonia is not a "spastic" disease. We identified patients with tremor (2), pyramidal and extrapyramidal disease (1), and myoclonic disorders (1). The remainder of the patients had clinical and EMG findings consistent with dystonia, a neurologic disorder characterized by abnormal, often action-induced, involuntary movements or uncontrolled spasms. We classify these patients as having "focal laryngeal dystonia" when the disorder occurs in isolation. It may also present as a component of a generalized dystonic syndrome.

Adult↗

Modified release tizanidine in the treatment of spasticity.

A 6-week study of a modified release formulation of tizanidine designed for once daily administration was performed in 27 patients with spasticity due to cerebral lesions. The dosage of tizanidine used ranged from 6 to 18 mg/day. At the start of the study all patients had at least moderate spasticity and 20 (74%) patients had severe or very severe spasticity. All had a decrease in muscle strength. After 1 week of treatment 22 (81%) patients showed improvement in overall spastic state and, after 6 weeks, all 27 patients had improved. At the end of treatment 25 (93%) patients showed an improvement in overall disability. The drug was well tolerated. Side-effects were reported in only four patients, and these were minor and mostly mild. Tizanidine had no clinically important effects on blood pressure, heart rate, body weight or laboratory values. Overall, once daily treatment with modified release tizanidine is well tolerated and gives good clinical efficacy in patients with spasticity.

Adult↗

Physical management of spasticity.

Physical management of people with spasticity is a complex task requiring the collaborative work of a multidisciplinary team. This article reviews some of the most commonly used treatment techniques and theories that lay the framework for the physical management of spasticity. Most crucial to the management of the person with spasticity is the skillful application of the techniques and theories discussed. Given the complexity in the standardized evaluation of children with spasticity, it is recommended that existing outcome measures or standardized scales be applied wherever possible as an adjunct tool to the clinician's assessment and interventions. One must recognize the benefits of such scales and their limitations. Therefore, it is recommended that further development of standardized assessments be supported by funding agencies to develop both quantitative and qualitative measurement tools that will address the particular needs of the person with spasticity. Review of the literature supports the many interventions that are listed; however, further research is recommended by the consensus group.

Adolescent↗

Use of modafinil in spastic cerebral palsy.

After an initial patient with cerebral palsy had an apparent dramatic reduction in spasticity when placed on modafinil, a pilot study was undertaken in 10 pediatric patients to confirm or refute the benefit of modafinil in cerebral palsy. Nine of 10 patients completed the 1-month treatment period. The study patients were treated with 50 or 100 mg of modafinil once daily in the morning. An assessment was made at baseline and at 1 month on treatment. All patients had a clinical examination, Modified Ashworth Scale scores (spasticity) determined by a physical therapist, and videotaping of ambulation. In comparing visit 1 (baseline) and visit 2 (on treatment), statistically significant improvement in the modified Ashworth Scale scores was noted in seven of the nine patients completing the study (P = .0080). A blinded review of the videotapes did not show statistically significant differences in ambulation, but the speed (ft/sec) of gait improved in six of the nine patients (P = .0192). In this study, modafinil, a newly released central stimulant for narcolepsy, showed benefit in treating spasticity in patients with cerebral palsy. A second larger, placebo-controlled, double-blinded trial is planned to confirm these initial results and observations. Modafinil appears to benefit spastic cerebral palsy by a yet to be determined mechanism; however, a primary effect of modafinil on brainstem structures is hypothesized to reduce spasticity of central origin.

Adolescent↗

Pelizaeus-Merzbacher disease and spastic paraplegia type 2: two faces of myelin loss from mutations in the same gene.

Pelizaeus-Merzbacher disease and X-linked spastic paraplegia type 2 are two sides of the same coin. Both arise from mutations in the gene encoding myelin proteolipid protein. The disease spectrum for Pelizaeus-Merzbacher disease and spastic paraplegia type 2 is extraordinarily broad, ranging from a spastic gait in the pure form of spastic paraplegia type 2 to a severely disabling form of Pelizaeus-Merzbacher disease featuring hypotonia, respiratory distress, stridor, nystagmus, and profound myelin loss. The diverse disease spectrum is mirrored by the underlying pathogenesis, in which a blockade at any stage of myelin proteolipid protein synthesis and assembly into myelin spawns a unique phenotype. The continuing definition of pathogenetic mechanisms operative in Pelizaeus-Merzbacher disease and spastic paraplegia type 2, together with advances in neural cell transplant therapy, augurs well for future treatment of the severe forms of Pelizaeus-Merzbacher disease.

Alleles↗

Atypical dementia and spastic paraplegia in a patient with primary lateral sclerosis and numerous necortical beta amyloid plaques: new disorder or Alzheimer's disease variant?

Primary lateral sclerosis (PLS) and hereditary spastic paraplegia (HSP) are clinically similar disorders in which progressive lower limb spasticity and corticospinal tract degeneration are characteristic. We report the occurrence of progressive spastic paraplegia and frontal systems dementia in a patient with postmortem features of PLS combined with moderate Alzheimer-like changes in neocortex and hippocampus. This combination of clinical and neuropathologic findings has not been described in PLS or HSP and varies from other cases in which spastic paraplegia, dementia, and Alzheimer neuropathology occurred concurrently. This 69-year-old woman developed spastic quadriplegia and dementia over 12 years. Left leg weakness progressed over 7 years to paraplegia, then quadriplegia by age 68. Sensory and cerebellar function were preserved and fasciculations were absent. Dementia characterized by concrete thinking, perseveration, and impaired executive function appeared in the seventh year and remained relatively stable until 6 months before death at age 69. Degeneration of the lateral corticospinal and dorsal spinocerebellar tracts confined to the spinal cord was evident at postmortem examination. Brain stem, midbrain, and cerebellum were normal. Numerous beta/A4 amyloid positive diffuse plaques (10-15/200x field) were apparent in neocortex, and neurofibrillary tangles immunopositive for paired helical filament were detected in hippocampus. This case broadens the spectrum of disorders associated with Alzheimer neuropathologic changes. The relationship between PLS, HSP, and Alzheimer's disease requires further study.

Aged↗

Guided intrathecal baclofen administration by using soleus stretch reflex in moderate-severe spastic multiple sclerosis patients with implanted pump.

We tested the hypothesis that changes in soleus stretch reflex was correlated to changes in intrathecal baclofen dose in 12 multiple sclerosis patients with moderate-severe spasticity treated with intrathecal baclofen pump. Twice patients were evaluated clinically and biomechanically. The short-latency soleus stretch reflex was elicited by rotating the ankle joint 4 degrees with a velocity from 3.1 to 180 degrees/s. There was a strong correlation between changes in intrathecal baclofen dose and amplitude of the short-latency stretch reflex (r=-0.88, P<0.001), which means that with an increase in baclofen dose there is a decrease in the amplitude. In contrast, no correlation exists between changes in intrathecal baclofen dose and clinical assessment of spasticity by using the Ashworth scale. The amplitude of the stretch reflex was very small (5 microV) compared with previous findings (>50 microV), which indicates an effective antispastic effect of intrathecal baclofen. We suggest that clinical evaluation of spasticity using Ashworth scale is insensitive to detect minor changes in moderate-severe spasticity and consequently might not be very useful in evaluating spasticity in relation to ambulatory filling of baclofen pumps. The soleus stretch reflex might be useful in situations when there is doubt about the effect of intrathecally administered baclofen.

Adult↗

Glycine and experimental spinal spasticity.

Spasticity may result in part from segmental spinal disinhibition. We determined the content and specific activity of glycine (the putative neurotransmitter thought to mediate spinal postsynaptic inhibition) and serine (the probable precursor of glycine) in feline spastic spinal cord following the intra-aortic administration of two labelled precursors of glycine--14C-D-glucose and 14C-L-serine. The specific activities of both glycine and serine were significantly reduced in the ventromedial, central, and dorsal spinal gray matter in spastic animals. Glycine content remained at control values but serine content increased in spastic spinal cord. This study suggests that glycine turnover decreases in spasticity, owing to its diminished release, and supports neurophysiologic evidence of a decrement in postsynaptic inhibition.

Animals↗

Circadian variation in plasma levels of free-form tissue factor pathway inhibitor antigen in patients with coronary spastic angina.

Tissue factor pathway inhibitor (TFPI) is known to inhibit the initial reaction in the tissue factor-mediated coagulation pathway. We measured plasma free-form TFPI antigen levels and monitored 24-h Holter recordings at 06.00, 14.00 and 22.00 h in 15 patients with coronary spastic angina, 13 patients with stable exertional angina, and 11 control subjects. There was a significant circadian variation in plasma free-form TFPI antigen levels in patients with coronary spastic angina (25.8+/-2.0 ng/ml at 06.00 h, 21.1+/-1.6 ng/ml at 14.00 h, and 20.2+/-1.4 ng/ml at 22.00 h; p<0.01). Furthermore, free-form TFPI antigen levels at 06.00 h were significantly higher in coronary spastic angina patients than in patients with stable exertional angina or control subjects (p<0.01). Free-form TFPI antigen levels increased after the ischemic attacks in coronary spastic angina (p<0.01). This circadian variation correlated with the frequency of attacks, with the peak level occurring between midnight to early morning in patients with coronary spastic angina.

Adult↗

Mechanical measures of spasticity in stroke.

Mechanical measurements of stretch reflexes show promise for the quantification of spasticity after stroke. Mechanical measurements can increase the objectivity, repeatability, and precision of spasticity quantification and provide additional insight into the neuropathology that is not available with current clinical measurements. Several techniques are reviewed and the advantages and disadvantages of quantification of spasticity using mechanical measurements are considered. Although mechanical measures of spasticity offer many advantages, much research needs to be done to establish a standard measurement of spasticity that can be easily implemented in the clinic.

Journal Article↗

The validity of electrophysiological criteria used in selective functional posterior rhizotomy for treatment of spastic cerebral palsy.

Although selective functional posterior rhizotomy (SFPR) is an established procedure for the treatment of spasticity, the electrophysiological criteria used to define which posterior rootlets should be cut have not been standardized. The purpose of this study was to determine the validity of the intraoperative electrophysiological criteria used to select posterior rootlets for sectioning in SFPR. Intraoperative stimulation of posterior lumbosacral nerve roots and rootlets, using a 50-Hz stimulus at threshold intensity, was performed in five nonspastic children (controls) undergoing laminectomy for spinal cord untethering and in 32 spastic patients undergoing SFPR. Electromyographic responses were recorded in the upper and lower limbs, the neck, and the face. The pattern of sustained responses was assessed in detail in 17 additional patients who had previously undergone SFPR and in the five controls. Sustained responses with ipsilateral lower limb extrasegmental spread occurred in both spastic patients and the control group. Contralateral lower limb spread and suprasegmental spread to the upper limbs, neck, and face were found only in spastic children. Sustained responses with an incremental pattern were restricted to the spastic population and correlated well with the extent of contralateral and suprasegmental spread, whereas decremental patterns were not associated with contralateral spread. It is concluded that contralateral and suprasegmental spread into the upper limbs, neck, and face, and incremental responses are probably valid criteria of abnormality.

Adolescent↗

Hip joint subluxation after selective dorsal rhizotomy for spastic cerebral palsy.

OBJECT: The effects of selective dorsal rhizotomy (SDR) procedures on the hip joints of children with spastic cerebral palsy (CP) are not well described. This study was performed to determine the incidence of hip subluxation in children with CP who underwent SDR at a single institution. METHODS: The study group comprised 82 patients (164 hip joints) with a mean follow up of 3.9 years. Forty-four patients had spastic diplegia (53.6%), 35 had spastic quadriplegia (42.7%), two had spastic triplegia (2.4%), and one had spastic hemiplegia (1.2%). The mean patient age at SDR was 5.2 +/- 2.4 years. Preoperative and postoperative hip radiographs were reviewed and the femoral head center edge (CE) angles were recorded. The mean pre- and postoperative right CE angles were 14.1 and 17.2 degrees, respectively, and those of the left were 13.6 and 15.1 degrees, respectively. Considering a change in CE angle greater than 5 degrees as clinically significant, 72 hips (43.5%) remained unchanged, 63 (38.4%) improved, and 29 (17.7%) worsened. Of a number of preoperative variables, including age at time of surgery, Gross Motor Function Classification System (GMFCS) level, ambulatory status, extent of hip subluxation, preoperative scoliosis, and asymmetry of hip subluxation, only GMFCS level was associated with worsening of hip subluxation. CONCLUSIONS: Selective dorsal rhizotomy is more likely to have a positive effect or no effect on hip joint subluxation rather than to have a negative effect. More severe involvement, as measured using the GMFCS, may predispose to worsening of hip subluxation after SDR.

Adolescent↗

[Clinical study of dantrolene sodium in the treatment of spastic and dystonic syndromes].

Dantrolene sodium has been given to 45 patients suffering from dyskinetic syndromes: 33 were suffering from spastic syndromes, either secondary to cerebral lesions at birth, or to other cerebral lesions, or to cord lesions; 9 were affected by infantile dystonic syndromes; 1 by dystonia muscolorum deformans and the last 2 patients were suffering from parkinsonism. The best dosage schedule was individual and ranged from 50 mg to 300 mg a day. In this range, the majority of the spastic patients showed reduction of spasticity, unrelated with the site of pathology: a slight one in 12 patients, a moderate one in 9 and a marked one in 2. On the contrary, slight improvement has been noticed in only two of the patients suffering from dystonic syndromes. In no case side effects has been noticed. In all patients who underwent slight or moderate improvement only, we tried to obtain better results on spasticity by growing the dosage schedules; but we have always noticed side effects, that is weakness or drowsiness and, sometimes, urinary uncontinence. Moreover 2 patients showed evidence of transitory metabolic side effects. Therefore our experience shows that dantrolene sodium is an useful drug into the therapy of spasticity, even if often a slight of moderate improvement only is achieved. Slow increase in dosage schedule, repeated laboratory controls and alternate periods of treatment and suspended treatment should be observed.

Adolescent↗

Effects of a single session of prolonged muscle stretch on spastic muscle of stroke patients.

The control of spasticity is often a significant problem in the management of patients with spasticity. The aim of this study was to evaluate the effect of a single session of prolonged muscle stretch (PMS) on the spastic muscle. Seventeen patients with spastic hemiplegia were selected to receive treatment. Subjects underwent PMS of the triceps surae (TS) by standing with the feet dorsiflexed on a tilt-table for 30 minutes. Our test battery consisted of four measurements including the modified Ashworth scale of the TS, the passive range of motion (ROM) of ankle dorsiflexion, the H/M ratio of the TS, and the F/M ratio of the tibialis anterior (TA). The results indicated that the passive ROM of ankle dorsiflexion increased significantly (p < 0.05) compared to that before PMS treatment. Additionally, PMS reduced motor neuron excitability of the TS and significantly increased that of the TA (p < 0.05). These results suggest that 30 minutes of PMS is effective in reducing motor neuron excitability of the TS in spastic hemiplegia, thus providing a safe and economical method for treating stroke patients.

Adult↗

[Appearance of spastic hip pain in cerebral palsy children].

UNLABELLED: Pain is a serious complication in the development of spastic dislocation of the hip joint, sometimes determining future of the patient, limiting the degree of his independence and comfort of his life. OBJECTIVES: Determination of 1. the frequency of appearance of lower limbs' pain among patients with subluxation and dislocation of the hip joint in the course of spastic form of cerebral palsy; 2. the influence of the surgical treatment on pain development. MATERIALS: We analysed 82 patients (126 spastic hips) treated between 1970 to 1995 with spastic form of cerebral palsy. Patients were divided into 3 groups according to the degree of hip instability and the performed method of treatment. Group 1-41 patients (77 spastic hips) without neurogenic instability of hip (mean value of migration percentage (MP) - 25%), group II - 14 patients (19 hips) with untreated dislocation of hip (MP - 100%), group III- 25 patients (30 hips) with dislocation and subluxation of hips treated by open reduction combined with Dega's pelvic osteotomy and intertrochanteric varus osteotomy of femur. METHODS: Each patient underwent clinical and radiological evaluation of hips. We assessed the value of migration percentage according to Reimers. We adopted the scale of intensity of pain according to Barie: 1) no pain, 2) sporadic pains, 3) frequent pains retreating after administration of routine analgesic drugs, 4) frequent pains persisting after administration of typical analgesics. The evaluation of pains also comprised their localisation within the hip joint and the knee joint of the same limb (projected pains). RESULTS: Group I in 11.7% of hips occurred mild pain, in 20.8% light pain and in 67.5% pain didn't occur, there was no severe pain. In group II in 73.7% appeared severe and mild pain, in 10.5% light pain and in 15.8% pain didn't appear. In group III severe and mild pain occurred in 37%, light in 33% and no pain in 30%. In all groups appearance and severity of pain depends of the degree of hip dislocation (p < 0.01 to 0.001). Appearance and aggravation of hips' pain depends on the degree of decentration of the femur's head from the acetabulum, estimated by the migration percentage (MP). Treatment and prevention demands "aggressive" operative procedure to avoid expansion of the hip's pain. Surgery decreases the frequency and intensity of pain but doesn't make patient totally free from the risk of its appearance.

Adolescent↗

Correlation of knee extensor muscle torque and spasticity with gait speed in patients with stroke.

The purpose of this investigation was to determine in 17 stroke patients the correlation between two independent variables (knee extensor muscle torque [KET] and spasticity on the paretic side) and one dependent variable (gait speed). The patients had a mean age of 59 years; time since onset of first stroke was 51 days. Each variable was measured twice on each of two consecutive days. Both KET and spasticity were measured on a Cybex II dynamometer. Peak torque was measured during five-second maximal voluntary isometric knee extension efforts. Spasticity was defined by the relative angle of reversal (RAR) of the Cybex electrogoniometer curve obtained during pendulum tests. Gait speed was measured as patients walked 8.0 meters at their most comfortable speed while using their usual devices. The average of each day's KETs, RARs, and gait speeds was used in the calculation of Pearson product moment correlations. The correlations between KET and gait speed were significant (p less than .05) and were .574 (day 1) and .571 (day 2). The correlations between the RAR and gait speed were not significant (-.204 day 1 and -.262 day 2). All measurements had intraday and/or interday reliability coefficients (ICC 3,1) exceeding .930. The results of this study confirm that gait performance is correlated with measures of KET but not spasticity in stroke patients. Thus, at the knee, extensor muscle torque, unlike spasticity, appears to be justified as a practical and objective clinical measure.

Adult↗