81. DYSTONIA The drug of longest experience in all the dystonias and the most effective agent for idiopathic torsion dystonia (dystonia musculorum deformans). http://www.neuro.wustl.edu/neuromedical_treatment/dystonia.html

DYSTONIA The following drugs have been used, with variable and unpredictable success, often with undesireable side effects, in virtually all of the many generalized, segmental and focal syndromes........baclofen, clonazepam, diphenhydramine, trihexyphenidyl. Injection into the affected muscle of botulinum toxin type A (BOTOX) has been used extensively and successfully in all the syndromes. Duration of effect varies from 3-6 months. Some patients have received as many as 12 treatments in a 5 year period. The limitations of the procedure are obvious.....skilled practitioners not generally available, short duration of benefit, uncertainty regarding long term effects after many treatments. Baclofen Lioresal. Scored tab 10, 20 mg. Much less often effective in dystonia than in spasticity due to pyramidal tract pathology. Initial dose 10 mg bid or tid, may be increased to as much as 80-120 mg od in divided doses. Clonazepam Klonopin. Scored tab 0.5, 1, 2 mg. Initial dose 0.5-1 mg od or bid, may be increased, slowly, to as much as 20 mg od in divided doses. Most patients cannot tolerate more than 4-8 mg od because of drowsiness. Diphenhydramine Benadryl.

82. Parkinson's Disease | Parkinson Society Canada It may be a hereditary and progressive disease that appears in childhood. This is called Generalized Dystonia (dystonia musculorum deformans). http://www.parkinson.ca/pd/nd_cond.html

Parkinson's Disease Newly Diagnosed Early Diagnosis and Medical Treatment Living with Parkinson's Conditions Confused with PD Conditions Resembling PD Initially Parkinson's Disease may be confused with a number of other conditions, including: Benign Essential Tremor (Familial Tremor) A common condition that may appear early or later in life and slowly progresses over time. The tremor is usually equal in both hands and increases when the hands are moving. The tremor may involve the head but spares the legs. Patients with Benign Tremor have no other Parkinsonian features and there is usually a family history of tremor. Parkinsonian Tremor and Benign Tremor generally respond to different drugs. A small number of patients with Benign Essential Tremor (less than five percent) develop PD. Shy Drager Syndrome A condition in which the earliest and most severe symptoms are those of insufficiency of the Autonomic Nervous System:- dizziness on standing, bladder difficulties and impotence. These autonomic symptoms are followed by PD symptoms such as rigidity, tremor, bradykinesia, postural instability and gait difficulty. There is some question among neurologists as to whether Shy Drager Syndrome is a form of PD or a separate disease.

83. NeuroCAST - Sessions Primary dystonia (previously called idiopathic dystonia or dystonia musculorum deformans) is often hereditary, although sporadic forms may occur. http://www.neurocast.com/site/content/sessions_Dystonia.asp

Dystonia is a neurological movement disorder characterized by sustained muscle contractions that often induce uncontrollable twisting or repetitive movements, and abnormal postures and positions. The disorder may affect the entire body or only a selected part of it, such as the eyes, neck, arms, or legs. Dystonia may also be associated with pain. It tends to consistently affect the same groups of muscles, thus producing rather predictable movements over time. Initially, dystonia tends to be precipitated by specific movements or tasks, though later it can be activated by sustained movements, and in advanced stages can be present at rest. Symptoms may arise as a result of dysfunction of the basal ganglia or thalamus, parts of the brain responsible for the modulation of movement. Because of the complexity of the condition, it may be misdiagnosed as other disorders, such as stress, stiff or "wry" neck, or a psychogenic disorder. In fact, dystonia is one of the most common movement disorders. According to the Dystonia Medical Research Foundation: Dystonia is estimated to be six times more prevalent than Huntington's Disease, ALS, or Muscular Dystrophy . . . yet as few as five percent of the over 300,000 persons in North America estimated to be affected have been correctly diagnosed.

84. A Couple More ?'s read about what is now being referred to as primary generalized dystonia, what used to be called idiopathic torsion dystonia or dystonia musculorum deformans. http://www.medhelp.org/forums/neuro/archive/13275.html

Questions in The Neurology Forum are being answered by doctors from The Cleveland Clinic , consistently ranked one of the best hospitals in America. Subject: a couple more ?'s Topic Area: Dystonia Forum: The Neurology and Neurosurgery Forum Question Posted By: anne on Monday, December 07, 1998 1) All the Drs I have seen have said that I would not be able to take Klonpin b/c of its sedating effect - is there something else like it that would not have the same effect (I did take it for a while when a dr thought I was having seizures - which was wrong)? 2) I forgot to list that I had taken Artane. I was very very nauseated and dizzy. I was also told that it affected short term memory which would never work for me being in school. Is there something else like it w/ side effects that are not as severe? 3) Does my dystonia appear to be like the generalized kind that progresses or is it mulitfocal? I've heard both of these from neurologists I've seen who are supposedly familiar with dystonia and botox. I can feel my back starting to tighten (but not as severe as my neck or legs). I assume this is a follow-up on my answer yesterday to questions posted by "Anne" about CP and dystonia. Just so you'll know (and others reading, too): for additional questions like this (that is, not a new topic) it is best to use the "post follow-up" button. That way, it's all together in one chain.

85. Humangenetik Bochum - Template_e Humangenetik Bochum » Services » Diagnostics » Molecular Genetics » dystonia musculorum deformans (DYT1). dystonia musculorum deformans (DYT1). http://www.ruhr-uni-bochum.de/mhg/LEISTUNGEN/DIAGNOSTIK/diagnostik-molgen-dyt1_e

Startseite A-Z Suche Kontakt Humangenetik Homepage Huntington-Center Research Groups Publications Services Diagnostics Councelling Relationship analysis/ Forensic Education Lecture Training Seminars Staff Workgroups Index GenLinks Research Journals Companies Institutes Contact Adresses How to find us Humangenetik Services for: Students Scientists Patients Doctors ... Dystonia musculorum deformans (DYT1) Dystonia musculorum deformans (DYT1) Details exclusively available in german version. Contact Info Dr. med. S. Wieczorek Tel. +49 234 3223831 see german version head

86. Evaluation And Treatment Of Dystonia Generalized dystonia (as in idiopathic torsion dystonia, formerly known as dystonia musculorum deformans) refers to dystonic involvement of both legs and at http://www.medscape.com/viewarticle/410595_3

87. Cahaba GBA - 172 - Stereotactic Pallidotomy And Thalamotomy Iacono RP, et al. Simultaneous Bilateral Pallidoansotomy for Idiopathic dystonia musculorum deformans. Ped Neurol, 1996; 14145148 (Abstract). Laitinen, LV. http://www.gamedicare.com/policies/172.htm

172 - Stereotactic Pallidotomy and Thalamotomy Subject: Stereotactic Pallidotomy and Thalamotomy Policy Number: Description: Stereotactic pallidotomy and thalamotomy are surgical techniques used in the treatment of severe Parkinson's disease and other movement disorders, including dystonia and tremor. Pallidotomy and thalamotomy are not new procedures, having been performed first in the 1950's. In the 1950's and 1960's, Cooper, Hassler, Leksell and others pioneered the development of these procedures. Thalamotomy was considered more effective than pallidotomy for tremor and dystonia, although less effective for akinesia and bradykinesia, and emerged as the more common surgical procedure for the treatment of Parkinson's disease. With the availability of levodopa for Parkinson's disease in the early 1960's, interest in pallidotomy and thalamotomy waned. Policy Type: Local medical necessity and coverage policy HCPCS Section Benefit Category: Surgery HCPCS Codes: 61720: Creation of lesion by stereotactic method, including burr hole(s) and localizing and recording techniques, single or multiple stages, globus pallidus or thalamus. HCFA'S National Policy: Not applicable Indications And Limitations Of Coverage And/Or Medical Necessity: In order for Medicare Part B to provide coverage for pallidotomy or thalamotomy for Parkinson's disease, the following criteria must be satisfied:

88. Dystonias Fact Sheet Torsion dystonia, previously called dystonia musculorum deformans or DMD, is a rare, generalized dystonia that may be inherited, usually begins in childhood http://www.ninds.nih.gov/health_and_medical/pubs/dystonias.htm

National Institute of Neurological Disorders and Stroke Accessible version Science for the Brain The nation's leading supporter of biomedical research on disorders of the brain and nervous system Browse all disorders Browse all health organizations More about a disorder Studies with patients Research literature Press releases Search NINDS... (help) Contact us My privacy NINDS is part of the National Institutes of Health The Dystonias Fact Sheet Get Web page suited for printing Email this to a friend or colleague Request free mailed brochure Table of Contents What are the dystonias? What are the symptoms? How are the dystonias classified? What do scientists know about the dystonias? ... Where can I get more information? What are the dystonias? The dystonias are movement disorders in which sustained muscle contractions cause twisting and repetitive movements or abnormal postures. The movements, which are involuntary and sometimes painful, may affect a single muscle; a group of muscles such as those in the arms, legs, or neck; or the entire body. Those with dystonia usually have normal intelligence and no associated psychiatric disorders. What are the symptoms?

89. Karger Publishers External Resources 8 Iacono RP, Kuniyoshi SM, Lonser RR, et al Simultaneous bilateral pallidoansotomy for idiopathic dystonia musculorum deformans. http://content.karger.com/ProdukteDB/produkte.asp?Aktion=ShowFulltext&ProduktNr=

90. Dystoniegeneral Translate this page est le lien dans les dystonies DYT 1, entre une mutation dans la séquence codant pour la torsine A et le tableau clinique de dystonia musculorum deformans. http://perso.wanadoo.fr/jeanpierre.gadbois/dystoniegeneral.htm

Génétique des dystonies primaires (Dr Marie-Hélène MARION) (Son intervention à Limoges le 23 avril 1999) Les dystonies d'origine génétique ont longtemps été considérées comme représentant une minorité de cas de dystonie, s'appliquant essentiellement aux formes familiales. Il y a dix ans l'équipe d'Ozelius aux USA localisait le gène DYT 1 de la dystonie généralisée primaire sur le chromosome 9, puis quelques années plus tard, identifiait la mutation de la maladie. Depuis, la connaissance de la génétique des formes généralisées et focales progresse à grands pas, conduisant à une nouvelle classification des dystonies et peut-être un jour à un traitement spécifique de ces dystonies. Les dystonies primaires se distinguent des dystonies secondaires, pour lesquelles une cause telle une maladie métabolique est à l'origine. Les dystonies généralisées de l'enfant se distinguent cliniquement des dystonies focales (restant localisées à un groupe musculaire) de l'adulte. Les progrès de la génétique permettent d'affiner cette classification. La dystonie généralisée primaire : Appelée "dystonia musculorum déformans" depuis la description par Oppenheimer au début du siècle, correspond à la dystonie liée au locus DYT 1 (chromosome 9), de transmission autosomale dominante à pénétrance réduite : la pénétrance réduite signifie que les porteurs du gène DYT 1 ne sont affectés par la dystonie que dans 30 % des cas environ. Ainsi, un enfant atteint a le plus souvent des parents n'ayant pas la maladie, alors que l'un d'eux est porteur du même gène que l'enfant. La dystonie primaire DYT 1 se caractérise par une dystonie débutant dans l'enfance au niveau d'un membre (le plus souvent un pied équin) et extension fréquente et rapide en moins de cinq ans aux autres parties du corps pour aboutir à une dystonie généralisée.

91. Dorlands Medical Dictionary Cf. myotonia and paratonia. dystonia defor¢mans progressi¢va, d. musculorum deformans. torsion dystonia, d. musculorum deformans. http://www.mercksource.com/pp/us/cns/cns_hl_dorlands.jspzQzpgzEzzSzppdocszSzuszS

92. Roche Lexikon Medizin (4. Aufl.) - Dystonie, Dystonia Translate this page Dys tonie, Dys tonia. engl. dystonia. Störung eines natürlichen Spannungszustandes (Tonus). D. musculorum deformans. idiopathische Torsionsdystonie. http://www.gesundheit.de/roche/ro07500/r8883.html

Dys tonie, Dys tonia engl.: dystonia D., kinetische engl.: spastic bradykinetic d. spastische Bradykinesie D., menstru elle engl.: dysmenorrhea D. musculorum deformans idiopathische Torsionsdystonie D., neurogenitale Pelipathia vegetativa D., neuro vegetative engl.: neurovegetative d.; autonomic dysregulation Sympathikotonie Vagotonie ETENY D., neurozirkulatorische; D., vasomotorische D A C OSTA ... * Syndrom Verwandte Themen Bradykinesie Da Costa* Syndrom Dysphagia, Dysphagie Pelipathia vegetativa ... vegetativ

93. Characterization Of The Rat Mutant Dystonic (dt): A New Animal Model Of Dystonia All Previous Aging Related Articles. Online Medical Dictionary. National Library of Medicine's PubMed directory of MEDLINE citations. Characterization of the rat mutant dystonic (dt) a new animal http://www.arclab.org/medlineupdates/abstract_6470761.html

Aging Research Center Home Page All Previous Aging Related Articles On-line Medical Dictionary National Library of Medicine's PubMed directory of MEDLINE citations. Characterization of the rat mutant dystonic (dt): a new animal model of dystonia musculorum deformans. - Lorden JF, McKeon TW, Baker HJ, Cox N, Walkley SU J Neurosci 1984 Aug;4(8):1925-32. An inherited neurological disorder characterized by sustained twisting movements during waking has been discovered in Sprague-Dawley rats. The mutation follows an autosomal recessive pattern of inheritance and has been named dystonic (dt). The rat mutants are indistinguishable from normal littermates in open field behavior and climbing activity prior to postnatal days 9 to 10. Clinical signs begin to appear on day 10 and include twisting of the axial musculature, hyperflexion of the trunk, self-clasping of forelimbs and hindlimbs, and poor placement of the limbs during locomotion. No morphological lesions of neural or non-neural tissues have been observed with routine light microscopy.

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