Athetosis and Dystonia

2020 ◽  
pp. 105-136
Author(s):  
Hiroshi Shibasaki ◽  
Mark Hallett ◽  
Kailash P. Bhatia ◽  
Stephen G. Reich ◽  
Bettina Balint

Athetosis is characterized by irregular, slow, writhing, bizarre movements seen in hands and feet. Athetosis is classified as minor athetosis and major athetosis based on its magnitude. Minor athetosis is seen in patients with mild cerebral palsy. Major athetosis is caused by organic lesions of the striatum, including cerebral palsy, as a residual state of encephalitis, and after anoxic encephalopathy. In these conditions, athetosis is commonly seen in combination with dystonia. Dystonia is characterized by sustained or intermittent muscle contractions causing abnormal, often repetitive movements or postures or both. In many cases of dystonia, the affected part of the body shows an abnormal writhing posture in the resting condition, and slow, writhing involuntary movements are superimposed on the abnormal posture with voluntary movement. For childhood-onset patients, dystonia is most commonly generalized; in adults, typically there is focal dystonia. Focal dystonia includes blepharospasm, cervical dystonia, writer’s cramp, and musician’s cramp. It is often task-specific and can be treated with local botulinum toxin injection. Generalized dystonia is caused by long use of neuroleptic drugs (tardive dystonia) and a variety of neurodegenerative diseases.

2005 ◽  
Vol 12 (2-3) ◽  
pp. 205-210 ◽  
Author(s):  
G. Porro ◽  
D. van der Linden ◽  
O. van Nieuwenhuizen ◽  
D. Wittebol-Post

Introduction: Deficient postural control is one of the key problems in cerebral palsy (CP). Little, however, is known about the specific nature of postural problems of children with CP, nor of the relation between abnormal posture and dysfunction of the visual system.Aim of the study: To provide additional information on the association of abnormalities in postural control and visual dysfunction of the anterior or posterior part of the visual system.Methods: Data resulting from ophthalmologic, orthoptic, neurological, neuro-radiological, and ethological investigations of more than 313 neurologically impaired children were retrospectively analyzed.Results: Abnormal postural control related to ocular and ocular motor disorders consisted of anomalous head control and subsequent abnormal head posture and torticollis. The abnormal postural control related to retrochiasmatical damage of the visual system consisted of a torticollis combined with adjustment of the upper part of the body, as if at the same time adapting to a combination of defects and optimizing residual visual functions.Conclusion: Visual dysfunctions play a distinct role in the postural control of children with CP.


1989 ◽  
Vol 98 (2) ◽  
pp. 93-97 ◽  
Author(s):  
Andrew Blitzer ◽  
Paul E. Greene ◽  
Mitchell F. Brin ◽  
Stanley Fahn

Dystonia is a neurologic disorder characterized by abnormal, involuntary movements causing twisting and turning postures; it is postulated to be a disorder of central motor processing. The dystonias, when classified by region of the body involved, have been characterized as focal, segmental, and generalized. Focal dystonia can affect jaw mechanics, leading to forceful contraction of the jaw muscles and resulting in inappropriate deviation of the jaw. Localized injections of botulinum toxin have been used successfully in the management of other focal or segmental dystonias. We have treated 20 oromandibular dystonia patients with botulinum toxin. Six patients had only jaw and tongue involvement; 11 had blepharospasm and jaw involvement; and three had jaw involvement as part of a more generalized dystonia. Five patients had been diagnosed originally and treated as having temporomandibular joint syndrome. All but one of the patients had improvement of their symptoms with the toxin injections. The patients averaged 47% improvement with the injections.


Toxins ◽  
2021 ◽  
Vol 13 (4) ◽  
pp. 278
Author(s):  
Dongwoo Lee ◽  
Jaewon Kim ◽  
Ja-Young Oh ◽  
Mi-Hyang Han ◽  
Da-Ye Kim ◽  
...  

We aimed to evaluate muscle mass changes after injection of botulinum toxin (BoNT) in children with spastic hemiplegic cerebral palsy (CP). Children aged between 2 and 12 years who were diagnosed with hemiplegic CP with spastic equinus foot were prospectively recruited and administered BoNT in the affected leg. Lean body mass (LBM) of both legs and total limbs was measured by dual-energy X-ray absorptiometry (DXA) preinjection and 4 and 12 weeks after injection. A total of 15 children were enrolled into the study. LBM of both legs and total limbs increased significantly over 12 weeks of growth. The ratio of LBM of the affected leg to total limbs and to the unaffected leg significantly reduced at 4 weeks after injection compared with preinjection but significantly increased at 12 weeks after injection compared with 4 weeks after injection. In conclusion, the muscle mass of the affected leg after BoNT injection in children with hemiplegic spastic CP decreased at 4 weeks after BoNT injection but significantly recovered after 12 weeks after injection.


2006 ◽  
Vol 86 (7) ◽  
pp. 987-1000 ◽  
Author(s):  
Chia-Ling Ho ◽  
Kenneth G Holt ◽  
Elliot Saltzman ◽  
Robert C Wagenaar

Abstract Background and Purpose. Children with cerebral palsy (CP) often are faced with difficulty in walking. The purpose of this experiment was to determine the effects of functional electrical stimulation (FES) applied to the gastrocnemius-soleus muscle complex on the ability to produce appropriately timed force and reduce stiffness (elastic property of the body) and on stride length and stride frequency during walking. Subjects and Methods. Thirteen children with spastic CP (including 4 children who were dropped from the study due to their inability to cooperate) and 6 children who were developing typically participated in the study. A crossover study design was implemented. The children with spastic CP were randomly assigned to either a group that received FES for 15 trials followed by no FES for 15 trials or a group that received no FES for 15 trials followed by FES for 15 trials. The children who were having typical development walked without FES. Kinematic data were collected for the children with CP in each walking condition and for the children who were developing typically. Impulse (force-producing ability) and stiffness were estimated from an escapement-driven pendulum and spring system model of human walking. Stride length and stride frequency also were measured. To compare between walking conditions and between the children with CP and the children who were developing typically, dimensional analysis and speed normalization procedures were used. Results. Nonparametric statistics showed that there was no significant difference between the children with CP in the no-FES condition and the children who were developing typically on speed-normalized dimensionless impulse. In contrast, the children with CP in the FES condition had a significantly higher median value than the children who were developing typically. The FES significantly increased speed-normalized dimensionless impulse from 10.02 to 16.32 when comparing walking conditions for the children with CP. No significant differences were found between walking conditions for stiffness, stride length, and stride frequency. Discussion and Conclusion. The results suggest that FES is effective in increasing impulse during walking but not in decreasing stiffness. The effect on increasing impulse does not result in more typical spatiotemporal gait parameters. [Ho CL, Holt KG, Saltzman E, Wagenaar RC. Functional electrical stimulation changes dynamic resources in children with spastic cerebral palsy. Phys Ther. 2006;86:987–1000.]


2020 ◽  
pp. 1-15
Author(s):  
Jūratė Lubienė ◽  
Dalia Pakalniškienė

The article presents the analysis of the onomasiological structure of the metaphorical somatonyms of Northern Samogitians, focusing on the indicators of the source of metaphors. Based on the explicit semantics of the source (lexical motivators), the metaphorical somatonyms of Northern Samogitians belong to several motivational models, the most productive of which is the artefactual motivational model. The basis of the artefactual metaphor is the associative similarity of the object (artefact) and the body part according to various parameters – shape, size, features of structure and materiality, actions, especially repetitive movements.


2020 ◽  
Vol 4 (Supplement_1) ◽  
Author(s):  
Azkia Khan ◽  
Hashim Ejaz ◽  
Vijaykumar Sekar ◽  
Sofia Syed

Abstract Background Acromegaly is a slow-onset rare endocrinopathy that is characterized by chronic overproduction of growth hormone (GH) and insulin-like growth factor-1 (IGF-1). Excess GH and IGF-1 levels lead to insulin resistance, which is implicated in most of the complications associated with acromegaly. We present a case of acromegaly, wherein the patient presented with worsening polyarthralgia and decreasing mobility. As the patient was undergoing workup for rapidly progressing osteoarthritis, the internist became suspicious of patient’s changing physical appearance. Case A 53-year-old man with medical history of diabetes mellitus and hypertension was referred to endocrine clinic on account of high IGF-1 levels, 909 ng/ml (normal: 37-245 ng/ml). He was seeking medical attention because of rapidly progressing polyarthralgia and stiffness for the last two years. His symptoms were intensifying despite use of non-steroidal anti-inflammatory drugs and intra-articular steroid injections. Imaging revealed severe degenerative changes and narrowing of joint space in bilateral hip, knee and glenohumeral joints. Internist observed that the patient was exhibiting stigmata of acromegaly such as enlargement of hands and feet, prognathism and dental space widening. Patient reported headaches, blurry vision, sleep apnea, dysphagia and right ear exostosis. Colonoscopy revealed hyperplastic polyps. Repeat IGF- 1 levels were 910 ng/ml (Normal: 37-245 ng/ml). Oral glucose tolerance test showed failure of suppression of GH. Serial GH levels were 4.50, 5.08, 6.74, 5.81 and 5.21 ng/ml (Normal: 0.01- 0.97 ng/ml). Tests for other endocrinopathies revealed the following results: serum prolactin 4 ng/ml (Normal <18 ng/ml), serum cortisol 7.9 ug/dl (Normal: 6-27 ug/dl), 24 hour urine cortisol 23mcg/24 hours (Normal: 3.5-45 mcg/24 hours), serum TSH 2.25 uIU/ml (Normal: 0.34- 3 uIU/ml), serum T4 level 0.7 ng/dl (Normal: 0.6-1.6 ng/dl) and serum T3 level 144.9ng/dl (Normal: 87-178 ng/dl). Serum total and free testosterone levels were 111 ng/dl (Normal: 240-950 ng/dl) and 3.89 ng/dl (Normal: 4.06-15.6 ng/dl) respectively and were suggestive of hypogonadism. MRI Brain showed 12x10x8mm pituitary adenoma. He was referred for transsphenoidal surgery for resection of pituitary adenoma. Conclusion Polyarticular osteoarthritis is an early manifestation of acromegaly. Systemic diseases associated with acromegaly are the primary reason for which most patients seek medical attention. It is important to look for coexisting endocrinopathies whenever the diagnosis of acromegaly is established, since mass effect of pituitary adenoma can wreak havoc on the endocrine system of the body. High index of suspicion, early diagnosis and prompt treatment are the key to reverse some but not all comorbid conditions associated with acromegaly.


2022 ◽  
Vol 5 (1) ◽  
pp. 01-04
Author(s):  
Aamir Jalal Al-Mosawi

Background: Cerebral palsy is a heterogeneous disorder that can cause a lifelong disability that is associated with a non-progressive damage in the brain. It is commonly caused by antenatal, perinatal, early postnatal and neonatal conditions. However, post-neonatal cases of acquired cerebral palsy have also been reported, and were commonly caused by infection. Patients and methods: The family of a girl from Qatar, who developed severe cerebral palsy caused by submersion injury, consulted us about the possible therapies for her condition. Clinical picture and brain imaging abnormalities are described, and the relevant literatures were reviewed with the aim of suggesting possible evidence-based therapies. Results: At the age of 23 months, a previously healthy girl developed anoxic encephalopathy after experiencing submersion injury. MRI showed evidence of significant hypoxic ischemic injury primarily affecting the deep grey matter, hippocami, mid-brain and the posterior cortex. EEG showed diffuse slowness of cerebral activity and diffuse attenuation of the background without no epileptic abnormalities suggesting diffuse encephalopathy resulting from diffuse cortical injury. At the about age of three and half years, her family consulted us about her condition as she was still showing no awareness to the environment, showing no significant spontaneous movements. She had poor head control. Unable to sit or stand alone, and had a flexed posture. She was on levetiracetam (Keppra), diazepam, and baclofen 30 mg daily. She was still having tracheotomy, and was fed through gastrostomy tube. Conclusion: In this paper, the rare occurrence of severe post-infantile cerebral palsy is described. Emphasis is made on the possibility of using evidence-based multi-factorial therapies in cerebral palsy.


Sensors ◽  
2020 ◽  
Vol 20 (24) ◽  
pp. 7312
Author(s):  
Julia Mazzarella ◽  
Mike McNally ◽  
Daniel Richie ◽  
Ajit M. W. Chaudhari ◽  
John A. Buford ◽  
...  

Perinatal stroke (PS), occurring between 20 weeks of gestation and 28 days of life, is a leading cause of hemiplegic cerebral palsy (HCP). Hallmarks of HCP are motor and sensory impairments on one side of the body—especially the arm and hand contralateral to the stroke (involved side). HCP is diagnosed months or years after the original brain injury. One effective early intervention for this population is constraint-induced movement therapy (CIMT), where the uninvolved arm is constrained by a mitt or cast, and therapeutic activities are performed with the involved arm. In this preliminary investigation, we used 3D motion capture to measure the spatiotemporal characteristics of pre-reaching upper extremity movements and any changes that occurred when constraint was applied in a real-time laboratory simulation. Participants were N = 14 full-term infants: N = six infants with typical development; and N = eight infants with PS (N = three infants with PS were later diagnosed with cerebral palsy (CP)) followed longitudinally from 2 to 6 months of age. We aimed to evaluate the feasibility of using 3D motion capture to identify the differences in the spatiotemporal characteristics of the pre-reaching upper extremity movements between the diagnosis group, involved versus uninvolved side, and with versus and without constraint applied in real time. This would be an excellent application of wearable sensors, allowing some of these measurements to be taken in a clinical or home setting.


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