Yonsei Medical Journal Vol. 34, No. 4, 1993 Delayed-onset Focal Dystonia After Stroke Young Chul Choi, Myung Sik Lee and Il Saing Choi The delayed-onset focal or segmental dystonia is a rare sequelae of cerebrovascular disease. The responsible lesion sites for the dystonia are variable and the pathogenesis is uncertain. This study reports three patients with delayed-onset focal dystonia as a complication of stroke. The interval between hypoxic insult and onset of dystonia were varied from 1 month to 1 year. Two adults and one child had focal lesions at the contralateral basal ganglia. The interval between the brain damage and dystonia did not appear to be related to the age at the time of hypoxic in- jury. The site of lesions may serve as an important factor in the pathogenesis. Key Words: Delayed-onset dystonia, stroke Dystonia is characterized by sustained mus- cle contraction, which leads to abnormal pos- ture, twisting and repetitive movements (Fahn, 1988). Since the first description by Hammond in 1871, delayed-onset dystonia is a rare but well known sequelae of cerebrovascu- lar disease (Dooling and Adams, 1975; Traub and Ridley, 1982; Demierre and Rondot, 1983; Russo, 1983; Obeso ef a/. 1984; Chiang and Lu, 1990). It may also develop as a sequelae of many other disorder such as; perinatal anoxia (Burke et al. 1980; Marsden ef al. 1985; Pettigrew and Jancovic, 1985; Saint-Hilaire et al. 1991), truama (Andrew e al. 1982; Brett and Hoart, 1981; Krauss et ai. 1992), tumor (Marsden e ai. 1985; Glatt and Nausieda, 1984; Narbona et al. 1984), infection (Dooling and Adams, 1975), vasculitis and SLE (Anegawa et al. 1986; Daras ef al. 1988). It can occur immediately after brain dam- age, but more frequently it develops after a variable period (Russo, 1983; Marsden ef al. 1985; Pettigrew and Jancovic, 1985). Caudate Recevied July 10, 1993 Department of Neurology, Youngdong Severance Hos- pital, Yonsei University College of Medicine, C.P.O. Box 8044, Seoul, Korea 120-752 Address reprint reguests to Dr. Y C Choi, Department of Neurology, Yongdong Severance Hospital, Yensei University Collegé of Medicine, Seoul, Korea Number 4 nucleus, lentiform nucleus, thalamus, internal capsule, or variable combinations of them have been reported as the responsible site for the delayed-onset dystonia (Marsden et al. 1985; Pettigrew and Jancovic, 1985). The pathologic process responsible for the dystonia during the initial recovery period is unknown. Some authors have compared the interval of delayed-onset dystonia in patients of perinatal anoxia and stroke. They empha- sized the age of hypoxic injury in deciding the duration of delay in spite of the differ- ences of etiology (Pettigrew and Jancovic, 1985). We present three patients with delayed- onset focal dystonia after stroke and re- viewed the related literatures. CASE REPORTS Patient 1 A 57-year-old male patient was referred due to painful paresthesia and dystonic pos- ture of the left hand. Five years ago, he suf- fered from a sudden onset of left side hemi- plegia and sensory loss. Brain CT scan showed an hemorrhage involving the right putamen, thalamus, internal capsule and head of the caudate nucleus (Fig. 1) The left 391 Young Chul Choi et al. hemiplegia improved enough so that he could walk without assistance several months later. Thereafter his left hand became clumsy. One year since the cerebral hemorrhage, dystonic posture in the left hand consisted of flexion of the metacarpal joints and extension of the interphalangeal joints began to appear. Re- petitive myorhythmic dystonic movement of the fingers were noticed. It consisted of slow Fig. 1. A brain CT of patient 1 showed hemorrhage in- volving the right putamen, thalamus, internal capsule and head of the caudate nucleus. A Fig. 3. The dystonic movement of patient 2 was sustained posture of dosiflexion(A) and was intensified by waking which made him walking discomfort(B). 392 spontaneous involuntary twisting, flexion of the metacarpophalangeal joints, fanning of the fingers, and extension of the thumb (Fig. 2) which was accentuated by stress. On ex- amination, mild dysarthria, left facial sensory loss and decreased gag reflex were noted. There was a mild hemiparesis with hyper- active deep tendon reflexes at the left side. There were definite deep and superficial sen- sory deficits in the left side. A routine hematological test, serum electrolyte, liver function test, and renal function test were normal. Fig. 2. Patient 1 had a spontaneous involuntary twist- ing movement of the left hand expressed as flex- ion of the metacarpophalangeal joints, and sepa- ration and extension of the thumb. B Volume 34 Fig. 4. Brain MRI of patient 2 showed an enlarged right frontal horn with high intensity changes involving the cau- date nucleus, lentiform nucleus, and cortex repetition time, 3000 msec; echo time, 80 msec). Patient 2 A 52-year old man was referred due to dystonic posture in the left limb. He had a history of hypertension and suffered a sud- den left hemiplegia 1 year ago. Examination at the time of admission revealed the left hemiplegia, dysarthria. Computed tomography of the head showed an infarction at the right middle cerebral artery territory. The left hemiparesis improved slowly and he was able to walk without support after several mon- ths. Nine months after the stroke he devel- oped dystonic posture of the left great toe. The posture consisted of sustained dorsiflex- ion, which was aggravated during walking (Fig. 3). On examination, mild dysarthria and left facial weakness were noted. Questionable hemiparesis with hyperactive deep tendon re- flexes was noted in the left limbs. Deep and superficial sensory deficits were noted in the left side. A routine hematological test, serum electrolytes, liver function test, and renal function test were normal. Serology for syphilis was negative. A Brain MRI showed an enlarged right frontal horn with high in- tensity changes involving the caudate nucle- us, lentiform nucleus, and cortex (Fig. 4). Patient 3 A 16-year-old boy was referred for the Number 4 Fig. 5. The left foot of patient 3 exhibited an intermit- tent or continuous spontaneous dystonic spasm of plantar flexion and slight inversion of the toes, evaluation of the dystonic posture of the left leg. He suffered from sudden weakness of the left leg when he was 7 years old, diag- nosed as cerebral infarction at that time. Along with the recovery over the next month, dystonic posture in left foot devel- oped gradually. He had no family history of neurological disease or medical illness. There was no specific findings during pregnancy and delivery. His development has been nor- 393 Young Chul Choi et al. PL Fig.6. A brain CT scan of patient 3 revealed an area of low attenuation adjacent to the right internal capsule in the caudate nucleus with an enlarged right frontal horn. mal. On neurological examination, a very mild left leg monoparesis with hyperactive deep tendon reflex was noted. Deep and su- perficial sensation were normal. The left foot exhibited dystonic posture and intermittent spontaneous dystonic spasm, which consisted of plantar flexion and slight inversion of the toes (Fig. 5). Left limb muscle tone was in- creased. These dystonic posture and move- ments were aggravated by any attempted action of the foot, but it decreased markedly during relaxation and disappeared during sleep. A complete blood count, urinalysis, serum electrolytes, renal function tests, se- rum ceruloplasmin and copper level were normal. ANA and RF were negative. A brain CT scan revealed an area of low attenuation adjacent to the right internal capsule in the caudate nucleus with an enlarged right fron- tal horn (Fig. 6). Mild improvement of the dystonic posture was noted after adminis- tration of trihexyphenidyl. DISCUSSION Delayed-onset dystonia is a rare sequelae of 394 stroke (Traub and Ridley, 1982; Demierre and Rondot, 1983; Russo, 1983; Obeso et al. 1984; Chiang and Lu, 1990). The anatomical basis and pathogenesis of delayed-onset dystonia is uncertain. Mitchell (1974) suggested that the delay in the onset of hemichorea or athetosis following hemiplegia was caused by progres- sive changes in the original brain lesion. Burke (1980) had hypothesized that the mech- anism of delayed-onset dystonia occurring a year or more after the insult may be due to aberrant neuronal sprouting. Pettrigrew and Jankovic (1985) studied 22 patients of delayed- onset dystonia following variable causes. They found the mean interval in the seven patients who had brain lesions below the age of 7 was much longer than the other pa- tients, and postulated that the age of the pa- tient at the time of cerebral injury influ- enced the latency of dystonia from the acute brain damage. However, the hypothesis of Pettrigrew has limitations. Some patients with immediate onset could not be explained with neuronal regeneration. Therefore we re- viewed the literature to find the relationship between the age of the patients at the time of injury and the period of delay in stroke patients. 34 cases of cerebrovascular disorder (Table 1) have been reported. Dystonia fol- lowing stroke almost always appeared within 1-12 months (mean 6.5 months). There were four patients with contralateral basal ganglia lesions (Table 1, patient No. 6, 14, 31, 32), who had a short delay despite the early age of hypoxic insult (Demierre and Rondot, 1983; Pettrigrew and Jankovic, 1985; Anegawa et al. 1986). In our series, one patient with dystonia following stroke in young age had a shorter interval compared to other elderly patients with dystonia following stroke. It became clear that there was no definite relationship between the time at the age of hypoxic in- sult and the interval of appearance of dys- tonia following stroke. In contrast, Factor et al. (1988) described a patient with long de- layed-onset dystonia followed by embolic in- farction at the age of 18 months. However, the possibility cannot be excluded that de- layed-onset dystonia was caused by perinatal hypoxia due to fetal distress during delivery. The pathological lesions in the patient of delayed-onset dystonia following stroke have a variety of anatomical lesion sites. Of 34 pa- Volume 34 Delayed-onset Dystonia Table 1. Clinical characteristics and anatomical lesion in patients reported on the literature with poststroke dystonia (IC: internal capsule; Inf.; infarction) No. of Age at Age of Duration Site of Etiology Anatomical site Reference patient insult onset of delay dystonia & pathology Burke(1980) 1 65 66 lyr Lt arm 2 54 55 lyr Lt arm Grimes(1982) 3 32 32 6mo Lt hand 4 50 50 5mo Ltarm 5 60 60 6mo Ltarm Demierre 6 6 6 Imo Lt hemi. (1983) 7 17 17 Imo Lt hemi. Glatt(1984) 8 68 68 4mo Lt hemi. Obesso(1984) 9 22 22 2mo Lt hemi. Sunohara 10 61 6! 3mo Lt hemi. (1984) Burton(1984) 11 47 47 9mo Lt hemi. Traub(1982) 12 43 46 3yr Lt hand Russo(1983) 13 56 56 2mo LtU/E Pettigrew 14 2 2 Imo Rt hemi. (1985) 15 36 36 Imo Lt hemi. 16 61 61 6mo Lt hemi. 17 46 46 Imo Lt hand 19 72 72 Imo Lt U/E 20 22 22 imo Rt U/E 21 22 22 Imo Lt hemi. 22 52 52 Imo Rt hemi. Marsden 23 68 68 2mo Rt hemi. (1985) 24 21 21 2mo Lt hand 25 62 62 6mo Lt hand 26 60 60 9mo Lt hemi. 27 52 52 4mo Rt hemi 28 51 51 6mo Rt foot 29 49 50 lyr Lt hemi. 30 48 51 8yr Lt hemi. Anegawa 31 5 5 3wk = Rt hemi. (1986) 32 6 6 2mo RtL/E Daras(1987) 33 42 44 2yr Lt hemi. Chiang(1987) 34 74 74 6mo Rt hemi. Present case 1 53 54 lyr Lt hand 2 51 52 9mo Lt foot 3 7 7 Imo Lt foot Infarction Lt MCA territory (Lt MCA occlusion) Infarction Normal Migraine Rt. putamen, caudate, thalamus Infarction Rt putamen, thalamus, temporoparietal cortex Infarction Rt putamen, caudate, anterior IC, thalamus Infarction Rt putamen, IC, caudate Embolic Inf. Rt lentiform, caudate, IC Infarction Rt thalamus, occipital, temporal cortex Migraine Rt thalamus, striatum, IC Infarction Rt thalamus, IC Infarction Rt putamen, IC Infarction Rt lentiform nucleus Infarction Rt lentiform nucleus Infarction Lt striatum Infarction Rt basal ganglia Infarction Rt internal capsule Embolic Inf. Rt basal ganglia Infarction Rt basal ganglia Infarction Normal Migraine Rt internal capsule Hemorrahage Rt IC, basal ganglia Infarction Lt thalamus Infarction Rt thalamus Infarction Rt halamus Hemorrhage Rt thalamus Infarction Lt lentjform, caudate, IC Infarction Lt lentiform, caudate, IC Embolic Inf. Rt lentiform, caudate, IC Infarction Rt lentiform, caudate, IC Vasculitis. Inf. Lt putamen Vasculitis Lt putamen SLE, Inf. Rt putamen Infarction Lt thalamus Hemorrhage Rt putamen, thalamus, caudate, ic. Infarction Rt lentiform, caudate, cortex Infarction Rt caudate, IC tients reported in the literature, 21 (61.7%) had basal ganglia lesion, 13 (38%) internal capsule lesion, 11 (82%) thalamic lesion, 2 (6.8 %) cortical lesion and 2 (5.8%) without any le- sions. Damage to the neuronal circuit con- necting caudate, putamen, globus pallidus and thalamus (Pettigrew and Jancovic, 1985; Fross Number 4 et al. 1987) seems to be responsible for the dystonia following stroke. 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