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Ne-W Entity Or Variant of Preexisting Syndromes? J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.49.1.52 on 1 January 1986. Downloaded from Journal ofNeurology, Neurosurgery, and Psychiatry 1986;49:52-57 Paroxysmal myoclonic dystonia with vocalisations: ne-w entity or variant of preexisting syndromes? TODD E FEINBERG,* ARTHUR K SHAPIRO,t ELAINE SHAPIRO,t From-the Departments ofNeurology, * and Psychiatry and Tourette and Tic Laboratory and Clinic, t Mount Sinai School ofMedicine, New York, USA SUMMARY From among 1377 patients with movement disorders, four patients had an unusual movement disorder characterised by paroxysmal bursts of involuntary, regular, repetitive, rhythmic, bilateral, coordinated, simultaneous, stereotypic myoclonus and vocalisations, often associated with tonic symptoms, interference with voluntary functioning, presence of hyperactiv- ity, attention and learning disabilities, and resistance to treatment with haloperidol and other drugs. This symptom complex may represent a new disease entity, referred to here as paroxysmal myoclonic dystonia with vocalisations or a variant or combination of other movement disorders such as Gilles de la Tourette, myoclonic, or dystonic syndromes. Between 1965 and 1983 we have examined 1377 cusses the differential diagnosis. The possibility of aProtected by copyright. patients thought to have a-tic disorder. Although new diagnostic entity is considered as well as the most (82%) were diagnosed as having Gilles de la possibility that the symptoms represent a spectrum Tourette syndrome,' 23 31 (2.3%) patients were varying between or the coexistence of Gilles de la diagnosed as having an atypical movement disorder Tourette, myoclonic, and dystonic disorders. because they did not fulfill DSM I12 or other34 criteria for Gilles de la Tourette syndrome or any Methods other- known movement disorder. We recently reviewed the history, signs, symptoms and clinical Each patient had a comprehensive. two-hour diagnostic evaluation which included completion by the patient and course to ascertain whether there were common fac- review by AKS of a detailed Movement Disorder Ques- tors or similarities among these patients. Several tionnaire and previous medical and psychiatric records, patterns emerged suggesting new movement entities interview of the patient, spouse or parents, and screening or atypical forms of other movement disorders. This physical and neurological examination. The data derived paper focuses on one such entity referred to by us as from the Movement Disorder Questionnaire comprise paroxysmal myoclonic dystonia with vocalisations. demographic, family movement disorder and medical his- These patients had a movement disorder charac- tories, birth, developmental, and patient past and current medical histories, psychological, social, academic and terised by paroxysmal bursts of involuntary, regular, http://jnnp.bmj.com/ repetitive, rhythmic, bilateral, stereotypic and occupational functioning, and symptom and treatment his- severe myoclonus with vocalisations often associ- tories. Patients diagnosed as Atypical Movement Disorder did ated with tonic movements, and which interfered not fulfill criteria for any movement disorder known to us, with-voluntary behaviour. These patients also had such as Gilles de la Tourette syndrome; simple, chronic or mild attention deficit disorder, hyperactivity, learn- multiple motor tics; transient tic of childhood; focal, seg- ing disabilities, and resistance to treatment with mental of generalised torsion dystonia; stereotypic move- haloperidol. This paper describes the signs, symp- ment disorder; Huntington's chorea; tardive dyskinesia; myoclonic epilepsy; cough of adolescence or adulthood; toms-and clinical course for these patients and dis- on September 26, 2021 by guest. myoclonus; essential tremor; etc. The diagnosis required agreement by the three authors. The records of these Address for reprint requests: Arthur K Shapiro, MD, Mount Sinai patients were reviewed repetitively, and selected patients School of Medicine Tourette and Tic Laboratory and Clinic, were reinterviewed. In addition to from the Annenberg Building, Room 22-72, One Gustave L Levy Place, reconstructing New York, New York 10029, USA. patients history and records the onset, progression and nature of the abnormal movements, we had the opportun- Received 27 April 1984 and in revised form 29 January 1985. ity in two cases to videotape examples of the movements. Accepted 23 March 1985 We include here the results of the videotape analysis, in 52 J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.49.1.52 on 1 January 1986. Downloaded from Paroxysmal myoclonic dystonia with vocalisations 53 addition to historical accounts, because we feel the exact- typically involved a muscle group and its synergists, while ness of such an analysis supplements and at times exceeds tonic movements simultaneously involved agonists and what the patients are aware of or observers can record with antagonists. Movements were bilateral and of large amp- the unaided eye. litude, generally moving a limb through 900, or the head through 30°-40. Myoclonic jerks were seen in isolation or Case descriptons superimposed on tonic spasm of the same muscles. Move- ments of the arms were bilaterally synchronous or assyn- Case I is a 21-year-old white male. Family medical and chronous. The muscle paroxysms occurred synchronously movement disorder histories, perinatal period and with guttural grunts and nasal exhalations in a nearly one- developmental milestones are non-contributory. The first to-one relationship. Movements and vocalisations were symptom of a movement disorder began at age 8 years with highly regular, rhythmic, and stereotyped, and tended to a loud inspiratory and expiratory throat clearing that was be regularly repeating, in 2-5 minute bouts separated by a longer in duration than a, typical tic. A repetitive, rough, variable period from 1-20 minutes. Analysis of individual guttural, inspiratory coughing sound occurred at age 10, myoclonic jerks revealed an initial excursion of the head, followed at 14 by a repetitive tonic back-bending or opis- neck or limb of 125-150 ms duration with considerable thotonic movement, insertion of finger in the throat and consistency. The muscle group may be held tonically in this external throat touching. At age 18 he developed a tonic new posture for a variable period of time to a maximum of flexing of the arms at the-elbow with superimposed repeti- 1-2 s, or rapidly returned to its initial position and toni- tive myoclonic jerks, and a year later developed a tonic cally held, again for a variable period. The changing dura- movement of the neck to the right and horizontally with tion of the tonic postures gave the movements a somewhat superimposed repetitive myoclonic movements. Repetitive varying frequency of 1-3 Hz, though successive move- sounds included throat clearing, coughs, grunts, barks, ments tended to be regular. A typical movement might screams, and high-pitched spitting sounds. Occasional cop- involve sudden simultaneous opisthotonic arching of the rolalia was described as involuntary but was characterised back with the arms held in front bent at the elbow with by the repetitive and regular utterance of the same word rapid, regular, rhythmic and bilateral flexion and extension more slowly than is usual in Gilles de la Tourette syn- of the arms. A coarse 6-8 Hz tremor of the hands was drome. The symptomatology has a waxing, waning and noted with the arms in extension. The movements occurred Protected by copyright. fluctuating clinical course. Symptoms partially decrease in at rest and with action. They interfered with movement the presence of strangers and with non-anxious absorption and made some simple actions, such as wiping the nose, in a task in the manner of Gilles de la Tourette syndrome. nearly impossible. There were no stimulus triggers. But the movements, unlike those in Gilles de la Tourette Case 2 is a 10-year-old white girl. Family history of move- syndrome, were the sanw in school as in the presence of ment disorder, general medical history, perinatal history the family, could not be voluntarily stopped, and would and maturational milestones are all non-contributory. The interrupt non-anxious absorption in tasks such as building history of the movement disorder began at age 8 years with models, when objects would be dropped owing to the tonic squeezes of various muscle groups with superimposed spasms. The patient had unsuccessful therapeutic trials myoclonic jerks. Over the course of the next six months with haloperidol and other neuroleptics, reserpine, ben- and continuing to the time of examination the patient zodiazepines, sedative-hypnotics, antiseizure medications, demonstrated tonic twisting and rotation of the out- clonidine, clonazepam, psychotherapy, behavioural stretched hands with superimposed, regular, rapid, myoc- therapy, and relaxation therapy. The general physical lonic movements. She had tonic movement of the torso to examination was unremarkable except for a fine distal tre- either side with superimposed rapid clonic jerks. Myriad mor bilaterally on finger to nose test, slightly clumsy fine vocalisations occurred, including repeated (4-5 times) finger and rapid alternating movements, and one or two hicupping, sneezing and barking noises, and unintelligible beats of clonus at the ankles bilaterally. The patient had a sounds such as " hihiahiahia" and woody woodpecker normal EEG, CT scan and lumbar CSF. Copper studies noises. Sounds were
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