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Myoclonus in Adult Huntington's Disease

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Myoclonus in Adult Huntington's Disease 8 Sloan MA, Price TR, Randall AM, ec al. Inuacerebral hemor- trate that myoclonus can be a disabling but treatable rhage after a-PA and heparin for acute myocardial infarction: feature in a subset of patients with adult HD. the TIM1 I1 pilot and randomized trial combined experience. Stroke 199O;21:182 Patient Histories 9 Kase CS, ONeal AM, Fisher M, et al. Intracranial hemorrhage T.T. was first evaluated at age 28 years, 2 years after the after use of tissue plasminogen activator for coronary thrombol- onset of cognitive decline and incoordination. The diagnosis ysis. Ann Intern Med 1990;112:17-21 10. Cras P, Kawai M, Siedlak S, et al. Neutonal and microglial of HD was supported by findings of dementia and chorei- involvement in B-amyloid protein deposition in Alzheimer's dis- form movements, and a definite family history of adult onset ease. Am J Pathol 1990;137:241-246 dementia and chorea previously diagnosed as HD (Fig 1). 11. Khachamian ZS. Diagnosis of Alzheimer's disease. Arch Neu- Neuropsychometric testing revealed a full scale IQ (FSIQ) of rol 1985;42:1097-1105 72, and positron emission tomography (PET) demonstrated 12. Mark DB, Hlatky MA, OConnor CM, et al. Administration hypometabolism in the caudate nuclei. Ceruloplasmin, serum of thrombolytic therapy in the community hospital: established and urine copper levels, electroencephalogram (EEG), head principles and unresolved issues. J Am Coll Cardiol 1988;12: computed tomographic (CT) scan, and cerebrospinal fluid 32A-43A (CSF) analysis were normal. Serial examinations over the 13. Vinters HV. Cerebral amyloid angiopathy a critical review. next 5 years demonstrated only a mild cognitive decline until Stroke 1987;18:311-324 14. Ishii N, Nishihara Y, Horie A. Amyloid angiopathy and lobar he presented with a I-month history of uncontrollable brief, cerebral hemorrhage. J Neurol Neurosurg Psychiatry 1984; rapid jerking movements. Examination revealed frequent 47:1203-1210 generalized myoclonic jerks involving axial and appendicular muscle groups, exacerbated by any attempt at movement, and prominent rigidity. Benztropine mesylare and halo- peridol had been started after the onset of the myoclonus. These drugs were discontinued without clinical change. Myoclonus in Adult A head CT scan showed prominent caudate atrophy. An EEG demonstrated generalized bisynchronous polyspike Huntington's Disease waves, at times associated with myoclonic jerks, without loss of consciousness and enhanced by photic stimulation (Fig 2). Caryn M. Vogel, MD," Ivo Drury, MB, HCh," L. Cass Terry, MD, PhD,t The myoclonus, as recorded by surface electromyography and Anne B. Young, MD, PhD* over the right forearm, consisted of 40 to 80-msec bursts. The cortical component of short latency somatosensory evoked responses (SSERs) was not enlarged. An eccrine Two brothers with clinically definite adult Hunting- sweat gland biopsy for inclusion bodies, a periodic acid-Schiff ton's disease developed disabling myoclonus years after stain for inclusion bodies in lymphocytes, an ophthalmolog- the first signs of the disease. Their electroencepha- ical examination for a cherry red spot, and a lysosomal en- lograms were consistent with a primary generalized zyme screen were negative. He did not cooperate with for- epilepsy, although neither man had seizures. The rnyo- mal neuropsychometric testing. clonus was controlled with valproic acid therapy. Valproic acid was instituted daily with a pronounced re- duction in myoclonus and a return to his previous level of Vogel CM, Drury I, Terry LC, Young AB. function. Follow-up examination 5 months later revealed Myoclonus in adult Huntington's disease. rare myoclonus and no polyspike-wave activity on EEG. Ann Neurol 1991;29:213-215 W.T., the older brother ofT.T., was diagnosed with HD at age 30 years, 2 years after the onset of cognitive decline and involuntary movements. His initial evaluation was similar to Myoclonus is an uncommon clinical feature in Hunt- his brother's except for caudate atrophy on head CT scan. ington's disease (HD). The prominent movement dis- Five years later, his caretakers reported a progressive decline order in affected adults is chorea, whereas in juvenile in ambulation and recent onset of jerking movements that patients, the clinical picture is one of parkinsonism { 1, were worse on awakening. On neurological examination, any 2). Only 3 patients with myoclonus complicating adult attempt to walk brought out myoclonic movements, which HD have been reported [2-43, although the preva- were otherwise infrequent. An EEG showed bisynchronous lence of myodonus may be greater in patients with polyspike waves enhanced by photic stimulation. These ap- juvenile onset C1, 51. The patients described here illus- peared both independent of and concomitant with the myo- clonus. The cortical component of the SSERs was not en- larged. Skin and rectal biopsies for inclusion bodies were negative by light and electron microscopy. Repeat neuro- From the *Department of Neurology, Universiry of Michigan Med- ical School, Ann Arbor, MI, and the ?Department of Neurology, psychometric testing revealed a FSIQ of 64, with a pro- Medical College of Wisconsin, Milwaukee, WI. nounced decline in motor speed and dexterity. Five months Received Mar 19, 1990, and in revised form Jd 31. Accepted for after starting valproic acid therapy, he could walk unassisted; publication Aug 2, 1990. however, the remainder of his neurological examination, in- Address correspondcncc to Dr Vogcl, University of Michigan Med- cluding the chorea, was unchanged. Follow-up EEG con- ical Center, Department of Neurology, 1915 Taubman Center, tinued to show generalized polyspike waves. An EEG and 1500 East Medical Center Drive, Ann Arbor, MI 48109-0322. SSERs were normal in a clinically unaffected brother. Copyright 0 1991 by the American Neurological Association 213 failure syndrome [S-81). These brothers, however, lacked the inheritance pattern and clinical, laboratory, or pathological findings specific to these disorders. The autosomal dominant transmission, adult onset of cho- rea and dementia, and highly characteristic PET and CT findings strongly support HD as the clinical diag- nosis in this family and the previously reported patients. The electrophysiological features present in these patients are most consistent with reticular reflex myo- clonus, which has been characterized as a fragment of generalized epilepsy [9]. There was an identifiable EEG correlate in the form of generalized polyspike waves that was not time-locked to the myoclonus. The cortical component of the SSERs was normal. Al- Fig 1.Family pedigree. Solid black squares = definite Hunt- though these brothers did not have generalized sei- ingtoni- disease (HD);shaded squares = possible HD. zures, their EEGs were similar to those seen in patients with a primary generalized seizure disorder, and 2 of the 3 previously reported patients with adult HD with myoclonus had documented seizures. Seizures occur rarely in patients with adult HD, compared with mo- thirds of the patients with juvenile HD {If. Deficiencies in gamma-aminobutyric acid (GABA) activity may play a role in the pathogenesis of some types of myoclonus. Reduced levels of GABA are present in the CSF of patients with postanoxic myo- clonus and progressive myoclonic epilepsies compared with controls consisting of patients suffering from other neurological disorders [lo, 1l}. The photosensi- Pig 2. Generali~edpo~spie-uia~ledischarges accentuated by pho- tic stimulation in T.T. before treatment. tive myoclonus in the Senegalese baboon Pupio pupio can be blocked by gamma-acetylenic GABA and gamma-vinyl GABA, irreversible inhibitors of the Discussion degrading enzyme, GABA-transaminase [12). The These brothers are similar to the 3 previously reported manipulation of GABA activity in the contralateral patients with adult HD with myoclonus [a-41. In all striatum of rats results in sustained focal myoclonic these patients, myoclonus was precipitated by the initi- jerks [l3). Clonazepam and valproic acid, effective ation of voluntary movement and often appeared as treatments of myoclonus due to diverse pathologies, massive sudden jerks involving axial and appendicular exert their therapeutic effects at least in part by in- muscle groups, and was easily controlled by either val- fluencing the GABA system [14}. proic acid or clonazepam. Each patient inherited HD In patients with rigid or juvenile onset HD, the from his father, and the onset of symptoms appeared denervation in the substantia nigra pars reticulata is in the third or early fourth decade. profound fl5-j. Bilateral lesions of the reticulata or Hereditary syndromes of myoclonus, dementia, and inhibition of the reticulata neurons by GABA agonists chorea comprise a rare and heterogenous group of dis- attenuate kindled seizures in rats [16]. Stimulation of orders. In addition to HD, the differential diagnosis the substantia nigra pars reticdata neurons or injection includes storage disorders (e.g., Lafora body disease, of GABA antagonists lower the seizure threshold in a and neuronal ceroid lipofuscinosis and sialidosis), mi- similar animal model [17]. This loss of nigral inhibition tochondrial encephalomyopathies (e.g., myoclonic from the striatum may play a role in the susceptibility epilepsy and ragged red fibers, and "Tweed Ball" to myoclonus or seizures of some patients with HD. mitochondropathy), inborn errors of metabolism (e.g., If deficiencies in GABA are central to the patho- glutaric acidemia, sulfite oxidase
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