1/24. Real-time continuous visual biofeedback in the treatment of speech breathing disorders following childhood traumatic brain injury: report of one case.The efficacy of traditional and physiological biofeedback methods for modifying abnormal speech breathing patterns was investigated in a child with persistent dysarthria following severe traumatic brain injury (TBI). An A-B-A-B single-subject experimental research design was utilized to provide the subject with two exclusive periods of therapy for speech breathing, based on traditional therapy techniques and physiological biofeedback methods, respectively. Traditional therapy techniques included establishing optimal posture for speech breathing, explanation of the movement of the respiratory muscles, and a hierarchy of non-speech and speech tasks focusing on establishing an appropriate level of sub-glottal air pressure, and improving the subject's control of inhalation and exhalation. The biofeedback phase of therapy utilized variable inductance plethysmography (or Respitrace) to provide real-time, continuous visual biofeedback of ribcage circumference during breathing. As in traditional therapy, a hierarchy of non-speech and speech tasks were devised to improve the subject's control of his respiratory pattern. Throughout the project, the subject's respiratory support for speech was assessed both instrumentally and perceptually. Instrumental assessment included kinematic and spirometric measures, and perceptual assessment included the Frenchay dysarthria Assessment, Assessment of Intelligibility of Dysarthric speech, and analysis of a speech sample. The results of the study demonstrated that real-time continuous visual biofeedback techniques for modifying speech breathing patterns were not only effective, but superior to the traditional therapy techniques for modifying abnormal speech breathing patterns in a child with persistent dysarthria following severe TBI. These results show that physiological biofeedback techniques are potentially useful clinical tools for the remediation of speech breathing impairment in the paediatric dysarthric population.- - - - - - - - - - ranking = 1keywords = movement (Clic here for more details about this article) |
2/24. history of Joubert syndrome and a 30-year follow-up of the original proband.The 1960s were a period of great flowering in the recognition of neurologic disorders in children. The so-called ataxic cerebral palsies were an especially fertile field waiting for clarification. Congenital ataxia coupled with hyperpnea-apnea, abnormal eye movements, and retardation was identified as an autosomal-recessive syndrome eponimically associated with the senior author, Marie Joubert. The disorder, though rare, is increasingly recognized and a lay society dedicated to family support and research has been formed. In preparation for a recent symposium the original proband was re-examined 30 years later and the manifestations in adults clarified. Severe dysarthria was the most striking feature in this man, the hyperpnea-apnea had diminished, and the abnormal eye movements were less striking. Ataxia was still present but not severe. Poor judgment and borderline intelligence rounded out the clinical picture. Modern imaging has clarified, in part, the anatomic basis of this syndrome.- - - - - - - - - - ranking = 2keywords = movement (Clic here for more details about this article) |
3/24. Vagal and hypoglossal Bell's palsy.A 7-year-old boy was referred because of a sudden change to nasal speech, dysarthria for words with explosive consonants in speech, and nasal regurgitation of fluids. The symptoms arose over 1 week following a capricious episode of acute asthmatic bronchitis. Physical and neurologic examinations were normal except for a left deviation of the uvula, accompanied by a "curtain" movement of the posterior pharyngeal wall against the opposite side, and a left deviation of the protruded tongue. No vascular, traumatic, infectious, neoplastic, or neurologic causes could be identified. No therapy was administered. Full recovery occurred 4 months later. The diagnosis was idiopathic vagal and right hypoglossal nerve palsy (Bell's palsy).- - - - - - - - - - ranking = 1keywords = movement (Clic here for more details about this article) |
4/24. Life-threatening dystonia-dyskinesias in a child: successful treatment with bilateral pallidal stimulation.We report a 13-year-old boy who developed severe, refractory dystonia-dyskinesias as an abrupt worsening of a previously nonprogressive movement disorder. The movements became continuous, requiring artificial respiration and continuous sedation in the intensive-care unit. Various drugs and drug combinations failed to achieve control. The child was then treated successfully with bilateral pallidal (GPi) stimulation as shown in the videotape. Four months later and without medication, the boy regained autonomous gait and audible speech; his neurologic condition continues to improve.- - - - - - - - - - ranking = 2keywords = movement (Clic here for more details about this article) |
5/24. A case of spinocerebellar ataxia accompanied by severe involvement of the motor neuron system.We report a sporadic case of spinocerebellar ataxia accompanied by later but severe involvement of the motor neuron system. A 72-year-old man began to show ataxia and dysarthria at age 66 years. Neurological examinations revealed saccadic eye movement, slurred speech, truncal ataxia, pyramidal sign, and urinary disturbance. Neither history of alcoholism nor hereditary factors were found. He developed muscular atrophy of the lower and upper extremities and limb ataxia within three years. Superficial and deep sensations were diminished in both feet four years after onset. Thus, he presented with cerebellar ataxia, bulbar sign, upper and lower motor neuron symptoms, sensory disturbance, and autonomic sign after six years at age 72. The level of serum, creatine phosphokinase (CPK) was increased, and muscle biopsy showed marked neurogenic change. magnetic resonance imaging (MRI) revealed mild cerebellar and pontine atrophy. Although the combination of spinocerebellar ataxia and motor neuron disease is very rare, the present case suggests the inter-relation of the spinocerebellar and motor neuron systems, and presents peripheral neuropathy as a subtype of multisystem atrophy.- - - - - - - - - - ranking = 1keywords = movement (Clic here for more details about this article) |
6/24. A new neurological entity manifesting as involuntary movements and dysarthria with possible abnormal copper metabolism.A few patients with an affected CNS involving abnormalities in copper metabolism have been described that do not fit any known nosological entities such as Wilson's disease or Menkes' disease.Three sporadic patients (two men and one woman) were examined with involuntary movements and dysarthria associated with abnormal concentrations of serum copper, serum ceruloplasmin, and urinary copper excretion. The onset of neurological symptoms occurred at the age of 15 to 17 years. The common clinical symptoms were involuntary movements and dysarthria. The involuntary movements included dystonia in the neck, myoclonus in the shoulder, athetosis in the neck, and rapid orobuccal movements. The dysarthria consisted of unclear, slow, and stuttering speech. Two of the three patients did not have dementia. A cousin of the female patient had been diagnosed as having Wilson's disease and had died of liver cirrhosis. Laboratory findings showed a mild reduction in serum copper and ceruloplasmin concentrations, whereas urinary copper excretion was significantly reduced in all three patients. Two of the three patients showed a high signal intensity in the basal ganglia on T2 weighted brain MRI.In conclusion, the unique findings of involuntary movements, dysarthria, and abnormal serum copper and urinary copper concentrations suggest that the three patients may constitute a new clinical entity that is distinct from either Wilson's or Menkes disease.- - - - - - - - - - ranking = 415.01700084875keywords = involuntary movement, movement (Clic here for more details about this article) |
7/24. Choreoathetosis related to lithium intoxication.A 76-year old woman was admitted because of severe gait ataxia and dysarthric speech that had worsened over the last 24 h. The patient was initially suspected of having repeated transitory ischemic attacks (TIAs) as her daughter reported a similar episode that had happened 3 weeks prior to admission. The onset of spontaneous twisting, choreoathetotic movements of both hands and arms and worsening of symptoms one hour after admission together with a history of lithium therapy lead to the correct diagnosis and appropriate treatment.- - - - - - - - - - ranking = 1keywords = movement (Clic here for more details about this article) |
8/24. The human basis pontis: motor syndromes and topographic organization.Clinical-anatomic correlations were performed in 25 patients with focal infarcts in the basilar pons to determine whether pontine lacunar syndromes conform to discrete clinical entities, and whether there is topographic organization of the motor system within the human basis pontis. Twelve clinical signs were scored on a 6-point scale, neuroimaging lesions were mapped and defined with statistical certainty, and structure-function correlation was performed to develop a topographic map of motor function. Clinical findings ranged from major devastation following extensive lesions (pure motor hemiplegia) to incomplete basilar pontine syndrome and restricted deficits after small focal lesions (ataxic hemiparesis, dysarthria-clumsy hand syndrome, dysarthria-dysmetria and dysarthria-facial paresis). The syndromes are not absolutely discrete, and are distinguished from each other by the relative degree of involvement of each clinical feature. Structure-function correlations indicate that strength is conveyed by the corticofugal fibres destined for the spinal cord, whereas dysmetria results from lesions involving the neurons of the basilar pons that link the ipsilateral cerebral cortex with the contralateral cerebellar hemisphere. Facial movement and articulation are localized to rostral and medial basilar pons; hand coordination is medial and ventral in rostral and mid-pons; and arm function is represented ventral and lateral to the hand. Leg coordination is in the caudal half of the pons, with lateral predominance. Swallowing is dependent upon the integrity of a number of regions in the rostral pons. Gait is in medial and lateral locations throughout the rostral- caudal extent of the pons. Dysmetria ipsilateral to the lesion constitutes a disconnection syndrome, as it occurs when the hemipontine lesion is extensive and interrupts pontocerebellar fibres traversing from the opposite, intact side of the pons. The heterogeneity of manifestations reflects the well-organized topography of motor function in the human basis pontis, in agreement with the anatomic organization of the motor corticopontine projections in the monkey. Higher order impairments including motor neglect, paraphasic errors and pathological laughter result from rostral and medial pontine lesions, and may result from disruption of the pontine component of associative corticopontocerebellar circuits.- - - - - - - - - - ranking = 1keywords = movement (Clic here for more details about this article) |
9/24. On-line anosognosia: unawareness for chorea in real time but not on videotape delay.In hemiplegics, anosognosia (unawareness of deficit) rests on a mismatch between expected and actual movement: a feedback hypothesis emphasizes sensory deficits or neglect, a feedforward hypothesis postulates impaired intention to move. Anosognosia for other problems is less studied. The authors report a man without sensory deficits who was unaware of choreiform movements, except on videotape delay. The authors believe that a feed-forward mechanism underlies his "on-line" unawareness.- - - - - - - - - - ranking = 2keywords = movement (Clic here for more details about this article) |
10/24. The Canadian multicenter trial of pallidal deep brain stimulation for cervical dystonia: preliminary results in three patients.OBJECT: deep brain stimulation (DBS) of the globus pallidus internus (GPi) is beneficial for generalized dystonia and has been proposed as a treatment for cervical dystonia. The Canadian Stereotactic/Functional and movement disorders Groups designed a pilot project to investigate the following hypothesis: that bilateral DBS of the GPi will reduce the severity of cervical dystonia at 1 year of follow up, as scored in a blinded fashion by two neurologists using the Toronto Western Spasmodic torticollis Rating Scale (TWSTRS). Secondary outcome measures included pain and disability subscores of the TWSTRS, Short Form-36 quality of life index, and the Beck depression Inventory. methods: Three patients have undergone surgery in Calgary with a follow-up duration of 7.4 /- 5.9 months (mean /- standard deviation). One patient underwent inadvertent ineffective stimulation for the first 3 months and did not experience a benefit until DBS programming was corrected. All three patients had rapid response to stimulation, with the muscles relaxing immediately and abnormal movements improving within days. Total TWSTRS scores improved by 79%, and severity subscores improved significantly, from 15.7 /- 2.1 to 7.7 /- 2.9 (paired t-test, p = 0.02). pain and disability subscores improved from 25.5 /- 4.1 to 3.3 /- 3.1 (paired t-test, p = 0.002) and from 13.3 /- 4.9 to 3.3 /- 4.2 (paired t-test, p = 0.06), respectively. CONCLUSIONS: Although it is too early to reach broad conclusions, this report of preliminary results confirms the efficacy of DBS of the GPi for cervical dystonia.- - - - - - - - - - ranking = 1keywords = movement (Clic here for more details about this article) |
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