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Whole-Body Tremulousness Isolated Generalized Polymyoclonus

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Whole-Body Tremulousness Isolated Generalized Polymyoclonus ORIGINAL CONTRIBUTION Whole-Body Tremulousness Isolated Generalized Polymyoclonus Andrew McKeon, MB, MRCPI; Sean J. Pittock, MD; Graham A. Glass, MD; Keith A. Josephs, MST, MD; James H. Bower, MD; Vanda A. Lennon, MD, PhD; J. Eric Ahlskog, PhD, MD Background: Acquired generalized repetitive myoclo- duration was 1.8 years, and mean age at onset was 55 years. nus may be mistaken for tremor. Distinguishing myoc- Referral diagnoses or patient complaints were tremor, lonus has etiologic and therapeutic implications. tremulousness, or shaking in all but 5 patients. All the pa- tients had repetitive myoclonus of all limbs, impairing gait Objective: To describe isolated generalized polymyo- in 14 patients. Surface electromyography confirmed non- clonus and the outcomes of etiologic evaluations at the periodic muscle burst durations of less than 50 millisec- time of diagnosis. onds, typical of myoclonus. Clinical and serologic screen- ing for cancer and autoimmunity revealed metastatic breast Design: Computer search of the Mayo Movement Neu- cancer in 2 patients (1 positive for ganglionic acetylcho- rophysiology Laboratory database and medical records line receptor antibody) and antibody profiles implicating linkage system. neurologic autoimmunity in 3 patients (CRMP-5 IgG or neuronal voltage-gated potassium channel antibodies). Setting: Department of Neurology, Mayo Clinic. Medications known to occasionally trigger myoclonus (opi- oids, selective serotonin reuptake inhibitors, and a serotonin- Patients: Nineteen adults with generalized repetitive my- oclonus confirmed using surface electromyography (burst norepinephrine reuptake inhibitor) were being taken by 7 duration Ͻ50 milliseconds), and other neurologic fea- patients. Myoclonus resolved after discontinuation of se- tures minimal or absent. lective serotonin reuptake inhibitor therapy in 1 patient; drug discontinuation was declined and follow-up was in- Interventions: Treatment of myoclonus and underly- adequate in the other 6. ing causes. Conclusions: Isolated whole-body tremulousness should Main Outcome Measures: Clinical presentation and raise the suspicion of generalized polymyoclonus, con- underlying etiologies. firmed using routine surface electromyography. Recog- nition is important because the differential diagnosis in- Results: We identified 19 patients with isolated general- cludes autoimmunity and drug-induced myoclonus. ized polymyoclonus resembling whole-body tremor. On- set was most often subacute (12 patients), mean symptom Arch Neurol. 2007;64(9):1318-1322 N OUR ROUTINE MOVEMENT DISOR- However, when generalized polymyoclo- ders clinical practice, occasion- nus develops as an isolated syndrome, the ally patients have been referred absence of clinical context and the resem- with a diagnosis of an atypical gen- blance to tremor may be diagnostically chal- eralized tremor syndrome that is lenging. The distinction is important be- Iultimately recognized as repetitive low- cause the symptomatic treatments differ and amplitude myoclonus (polymyoclonus). adult-onset generalized myoclonus is typi- Author Affiliations: Generalized polymyoclonus may re- cally acquired and may have an autoim- Departments of Neurology semble tremor but is distinguished by the mune basis, including paraneoplastic, or (Drs McKeon, Pittock, Glass, absence of true periodicity, which can be may be drug induced.1 Herein we describe Josephs, Bower, Lennon, and confirmed using surface electromyogra- a series of adult patients with a syndrome Ahlskog), Immunology phy (EMG). Although this resemblance may of generalized repetitive myoclonus, most (Dr Lennon), and Laboratory Յ Medicine and Pathology occur in the context of a toxic-metabolic en- with recent onset (duration 2 years), con- (Drs Pittock and Lennon), cephalopathy or a neurodegenerative con- firmed by means of surface EMG. Tremor, Mayo Clinic, Rochester, dition, the accompanying clinical features tremulousness, or gait disorder was the typi- Minnesota. of these disorders facilitate recognition. cal referral diagnosis. (REPRINTED) ARCH NEUROL / VOL 64 (NO. 9), SEP 2007 WWW.ARCHNEUROL.COM 1318 ©2007 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 09/30/2021 Table 1. Patient Demographics and Clinical Characteristics Patient No./ Age at Symptom Onset of Diagnosis at Sex Symptom Onset, y Duration, mo Symptoms Referral Diagnosis Chief Complaint Initial Examinationa 1/F 73 18 Insidious Tremor Jerks Myoclonus 2/M 55 120 Insidious Involuntary movements Jerks Myoclonus 3/M 51 24 Insidious Gait disorder Jerks Myoclonus or tremor 4/M 66 4 Subacute Tremulousness Tremulousness Myoclonus or tremor 5/M 34 6 Subacute Involuntary movements Shaking Myoclonus or tremor 6/M 44 24 Subacute Tremor Tremulousness Tremor 7/F 69 6 Insidious Parkinsonism Balance problems Myoclonus 8/F 63 6 Subacute Dystonia, possibly nonorganic Loss of limb dexterity Myoclonus 9/M 33 18 Insidious Tremulousness Tremulousness Myoclonus 10/M 77 12 Subacute General functional decline Tremors Myoclonus 11/M 64 6 Subacute Gait disorder Tremors Tremor and myoclonus 12/F 23 12 Subacute Involuntary movements Shaking Myoclonus 13/F 30 6 Subacute Tremor Tremor Myoclonus 14/F 67 36 Insidious Tremulousness Tremulousness Myoclonus 15/M 77 36 Subacute Tremor Tremors Tremor 16/M 76 15 Acute Gait disorder Shaking Myoclonus 17/F 72 12 Subacute Tremor Tremors Myoclonus 18/F 48 Uncertain Subacute Tremulousness Tremulousness Myoclonus 19/F 22 24 Insidious Involuntary movements Balance problems Myoclonus a By a staff neurologist. METHODS tion of symptoms at the time of presentation was 21.3 months (range, 4-120 months). Myoclonus was not a re- We sought to capture data on adult patients with a syndrome ferral diagnosis in any of the 19 patients, all of whom had of acquired myoclonus affecting the whole body and develop- been seen by a neurologist before review at Mayo Clinic. ing in the absence of obvious systemic illness or other neuro- The chief complaints from the patients were tremors or logic disease. The specific clinical inclusion criteria were as fol- tremulousness (10 patients), shaking (3 patients), jerks lows: (1) a syndrome of generalized repetitive myoclonus (3 patients), and balance or dexterity problems (3 pa- confirmed by surface EMG; (2) other neurologic features mini- tients). A family history of myoclonus or dystonia or ame- mal or absent, except for impaired gait, balance, and dexterity lioration of symptoms with alcohol use was not re- directly attributable to the myoclonus; (3) absence of major or- corded in any case. gan failure; and (4) age at onset older than 21 years. The sur- face EMG criteria were 2-fold: (1) documentation of muscle All 19 patients were examined by a Mayo staff neu- burst durations less than 50 milliseconds and (2) absence of rologist. A movement disorders specialist examined all periodicity (tremor is, by definition, periodic). but 2 patients before the neurophysiologic assessment; We ascertained these cases by searching the Mayo Move- 13 had their condition evaluated by at least 1 of us. My- ment Neurophysiology Laboratory computerized database and oclonus (sudden, brief, shocklike, involuntary move- medical records linkage system (1998-2006). Patients with fo- ments) was identified on clinical examination in 13 pa- cal, segmental, epileptic, or propriospinal myoclonus were ex- tients, with 4 others being characterized as having cluded, as were those with coexisting movement disorders (dys- myoclonus and/or tremor; however, tremor was the di- tonia, tremor, or chorea). The neurophysiologic features were described and myoclonus was documented by movement dis- agnosis in 2 patients. In all of the patients, the observed orders neurophysiology staff in all cases. From this original co- tremulous movements affected all 4 limbs and were re- hort the medical records were then reviewed to exclude pa- petitive (generalized polymyoclonus), with upper limb tients not meeting the previously described criteria. Thus, predominance found in 2 patients. Cranial involvement patients with myoclonus occurring in the context of neurode- was documented in 3 patients, and it was sufficient enough generative disease, encephalopathy, epilepsy, or obvious toxic- to cause dysarthria in 2. Opsoclonus or palatal myoclonus/ metabolic disease were excluded. We retained patients with mi- palatal tremor was not present in any of the patients. In nor or incidental neurologic findings in the study. 14 of the 19 patients, gait and stance were impaired by the ongoing generalized polymyoclonus. This ranged from RESULTS subjective unsteadiness alone to severe gait instability; 1 patient experienced myoclonic knee buckling, ob- PATIENT DEMOGRAPHICS served by the physician. In 2 patients, the physician com- AND CLINICAL FEATURES mented that the myoclonic jerks in the lower limbs dur- ing standing were better appreciated by palpation than We identified 19 patients who met the previously men- inspection. Minor or incidental features noted on exami- tioned criteria of isolated generalized polymyoclonus; 10 nation were findings suggestive of small-fiber neuropa- were men (Table 1). The mean age at symptom onset thy (patient 3), mild cognitive impairment (patient 7), was 55 years (range, 22-77 years), and the mean dura- and reduced arm swing on ambulation (patient 15). (REPRINTED) ARCH NEUROL / VOL 64 (NO. 9), SEP 2007 WWW.ARCHNEUROL.COM 1319 ©2007 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 09/30/2021
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