LATE-ONSET POST-HEMIPLEGIC HEMIDYSTONIA: CT AND ANGIOGRAPHIC FINDINGS G. J. HANKEY Neurology Registrar, Royal Perth Hospital. W A M. SADKA Neurologist, Royal Perth Hosptal, WA Abstract: A 27-year-oldwoman is described who suffered an acute left hemiplegia at the age of three years and 20 years later she noted t h e onset of unilateral left limb dystonic movements. Her cranial CT scan showed an area of low density, consistent with longstanding infarction, in the right lentiform nucleus. Cerebral angiography demonstrated aneurysmal dilatation of the terminal portion of the right internal carotid artery, minor irregularity of the lenticulostriate branches of the right middle cerebral artery (suggestive of Moya Moya disease) and occlusion of the right anterior cerebral artery. The dystonic movements improved with levodopa therapy. Clinico-radiological correlation in this case supports recent evidence for a disruption of pathways between the caudate nucleus, lentiform nucleus and thalamus in the pathophysiology of hemidystonia. (Aust NZ J M e d 1989; 19: 147-150.) Key words: Hemidystonia, stroke, computerised tomography, cerebral angiography, levodopa. festation (from several days up to 29 years) of acute INTRODUCTION hemiplegia in ~ h i l d h o o d . ~Lesions .~ in the same Dystonia is a state of abnormal posture produced territory in adult life rarely produce dystonia.6-8 by slow, sustained involuntary muscle contractions with may affect a single muscle, a group of muscles Since the introduction of computerised tomography (CT) focal lesions have been identified in or most of the muscles of the body, resulting in the contralateral caudate nucleus, lentiform nucleus characteristic postures.' Although often classified (particularly the putamen), thalamus and anterior according t o its anatomical distribution (focal, limb of the internal capsule in patients with segmental, unilateral and generalised), dystonia is unilateral dystonia of vascular more practically classified according to etiology, as On the basis of anatomical findings, it is currently either primary (idiopathic) or secondary dystonia.' Most patients with dystonia have primary torsion postulated that hemidystonia is either a result of dystonia, which is either hereditary or idiopathic.* abnormal input from the thalamus to the premotor Less commonly, dystonia may be symptomatic of cortex and/or a disconnection of the pathways between the striatum and thalamus, with relative and secondary to perinatal cerebral lesions, preservation of the corticospinal pathway^.^,^ encephalitis, demyelinating disorders, trauma, However, the precise pathophysiology remains tumour, metabolic disorders, drugs and cerebrovascular d i ~ e a s e . ~ uncertain. Unilateral limb dystonia (hemidystonia), defined CASE REPORT as involuntary, sustained posturing of the unilateral A 27-year-old Vietnamese woman presented with a four year arm, leg and face is most frequently a manifestation hi5tory of progressive difficulty using her left limbs due to of cerebrovascular d i ~ e a s e . Hemidystonia ~ is involuntary sustained flexion and extension movements of the nevertheless a rare post-hemiplegic c o m p l i c a t i ~ n , ~ left toes, ankle and fingers. Although present at rest, her and when it occurs, is most often a delayed maniabnormal distal left limb postures were exacerbated by Reprint requests to: Dr Graeme J . Hankey, Department of Neurology, Mayo Clinic, Rochester, Minnesota 55905, USA. LATE-ONSET POST-HEMIPLEGIC HEMIDY STONlA Aust NZ J Med 1989; 19 147 Figure 2: Cranial C T scan with contrast showing a circular area of increased density (also seen o n plain CT) in the terminal right internal carotid artery. Figure I : Plain cranial C T scan showing an area of low density, consistent with infarction, in the right lentiform nucleus. Figure 4: Right ICA subtraction angiogram (lateral view) showing aneurysmal ectasia of the terminal right ICA and proximal occlusion of the right ACA. Figure 3: Right internal carotid artery (ICA) subtraction angiogram, A-P view, showing irregular dilatation of the terminal right ICA and minor irregularities of the lenticulostriate branches of the right middle cerebral artery with a Moya Moya-type ‘puff of smoke’ (arrow). The right anterior cerebral artery (ACA) fails t o opacify. 148 movement. In particular, walking precipitated severe flexor and extensor spasm of the left ankle and toes. At the age of three years she experienced an acute episode of moderate weakness in the left leg and, less so, left arm. This persisted and she had walked with a limp since that time. She had otherwise been well. Abnormalities o n physical examination were confined to the nervous system. Cranial nerve examination was normal. Examination of the outstretched upper limbs revealed slow, irregular flexion and extension movements of the left fingers at the metacarpophalangeal joints over a iange of approximately 20-30 degrees. Intermittent dyslonic posturing of the left foot was present. The predominant posture was one of flexion and inversion of the left ankle with fluctuating flexor and extensor posturing of all toes of the left foot. The dystonic posturing of Aust NZ J Med 1989; 19 EIANKEY AND SADKA the left foot was at times precipitated and exacerbated by walking. The left lower limb was three centimetres shorter than the right and considerable wasting of the posterior compartment of the left leg was present. Fasciculations were not present. Mild left sided hypertonia, hyperreflexia and weakness in a pyramidal distribution were present with a left extensor plantar response. The left abdominal reflexes were absent. Sensation was preserved. Routine hematological and biochemical investigation5 were normal. An X-ray of the pelvis revealed a reduction in size of [he entire left hemipelvis and some atrophy of the left femur and femoral head. Cranial CT scan without and with contrast showed a small low density area in the right lentiform nucleus consistent with infarction of longstanding (Fig. I ) . A rounded calcific opacity was present in the region of the terminal right internal carotid artery (ICA) (Fig. 2). Four vessel cerebral angiography disclosed irregular ectasia of the terminal right ICA which continued as the main t r u n k supplying the right middle cerebral artery (MCA). Minor irregularities were seen to affect the lenticulostriate branches of the right MCA at its origin (MI segment), suggestive of a mild form of Moya Moya disease (Figs. 3 , 4). The right anterior cerebral artery (ACA) was occluded proximally but opacified via injection of the lcft ICA. The patient’s left limb dystonia responded incompletely to levodopa 100 mg and benserazide 25 mg (Madopar M) one tablet twice daily. DISCUSSION The patient described suffered an acute left hemiparesis at the age of three resulting in a residual left hemiparesis of moderate severity with crural predominance and left facial sparing. Twenty years later, she noted the delayed onset of mild dystonia in the left hand and a moderately severe dystonia at the left ankle. From the angiographic findings, it is hypothesised that the terminal component of the right ICA dissected at the age of three causing secondary occlusion of the origin of the right ACA. A Moya Moya effect is seen o n the M1 segment of the right MCA with minor irregularities affecting the lenticulostriate branches of the right MCA. The C T appearances of infarction in the right lentiform nucleus are consistent with this hypothesis. Demierre and Rondot reported three cases of unilateral dystonia of vascular origin (during childhood) with CT evidence of ischemic lesions in the putamino-capsulocaudate region in the territory of supply of the lenticulostriate artery near its origin from the MCA.’ Marsden e t a / . reviewed 28 patients with focal or hemidystonia of whom 15 had a history of previous hemiparesis or hemiplegia due to cerebral infarction.’ The site of the responsible lesion, as defined by CT scan or pathological examination, was in the contralateral caudate nucleus, lentiform nucleus (particularly the putamen) or the thalamus, alone or in combination. Symptomatic focal foot dystonia was present in only one post-stroke case in whom CT disclosed thrombotic infarction in the lenticulo-capsulo-caudate regi01-1.~ Pettigrew and Jankovic studied 22 patients with hemidystonia and seven were due to cerebral infarction.‘ On CT evidence, lesions were present in the basal ganglia in six cases. The corticospinal tract was partially involved in the internal capsule in two patients, one of whom had infarction only in the genu of the contralateral internal capsule. Although there was no CT evidence of internal LATE-ONSET POST-HEMIPLt(;lC H E M I O Y S r O N l A capsule infarction in our patient, she, like other similar previously reported cases,4 ’ undoubtedly had clinical evidence of corticospinal tract dysfunction which was almost certainly at the level of the internal capsule. Although lesions of the caudate nucleus, lentiform nucleus, thalamus and anterior limb of the internal capsule which have occurred in childhood may subsequently induce dystonia, it is curious that ischemic lesions in the same territory occurring in adults only extremely rarely result in dystonia.’* The reason for this remains unclear and perhaps it is too simple to propose that children who suffer stroke experience delayed onset posthemiplegia dystonia only because they live longer than adults with stroke. It has been suggested by Burke et al., that perhaps delayed onset dystonia is the result of slowly evolving aberrant neuronal sprouting in the central nervous system, stimulated by the original static lesion.’z This concept has also been considered as a possible explanation for the predisposition to premature Parkinsonism in patients with pre-damaged nigro-striatal pathways. Perhaps the natural attrition of cells within the basal ganglia compounds upon the pre-existing damage and the specific clinical manifestations of the syndrome are determined by the site of the original lesion. This, of course, remains speculative. The presence of other disabilities such a5 hemiparesis may also mask signs of dystonia and delay its detection. From two recent reviews, which have identified lesions of the basal ganglia and/or thalamus in the majority of cases of hemidystonia, it is considered that disruption of the pathways between the striatum, pallidum and thalamus, with relative sparing of the corticospinal tract, is probably essential for secondary dystonia to occur.4 The caudate nucleus and putamen project topographically to the globus pallidus, which itself projects to the thalamus. The thalamus sends input to the premotor and motor cortex. Marsden e t a / . , believe that dystonia may result from disruption of thalamic input to the frontal cortex, and ultimately to the motor area, resulting in the delivery of inappropriate commands to the premotor and motor cortex.’ They therefore view dystonia as a consequence of release of premotor cortical activity from thalamic control. This postulate is supported by the rare but recognised occurrence of hemidystonia following stereotactic t h a l a m ~ t o m y and , ~ the pathological studies of four cases of severe post-hemiplegic dysonia by Dooling and Adams.5 In all cases the principal lesion lay in the striatum and more extensively in the putamen than the caudate. Loss of striopallidal and pallidothalamic fibres was present with retrograde cell degeneration in the thalamus.’ Symptomatic, almost focal, foot dystonia, as seen in our case, is much more uncommon than hemidystonia or clystonia o f the hand and arm but has been reported with lesions involving the caudate nucleus and/or lentiform nucleus. Although the response of dystonia to medicarions is generally disappointing we have been encouraged with the response to levodopa therapy in this patient. This paper presents a patient with delayed onset hemidystonia, affecting the left foot particularly, Aust N Z .I Med 1089; 19 I49 following the onset of hemiparesis in childhood associated with a structural vascular lesion in the contralateral lentiform nucleus. Angiography demonstrated aneurysmal dilatation of the terminal contralateral ICA and irregularities of the lenticulostriate branches of the right MCA. Clinico-radiological correlation in this case further supports the accumulating evidence for a disruption of pathways between the contralateral caudate nucleus, lentiform nucleus and thalamus in the pathophysiology of hemidystonia. Treatment with levodopa should be considered as a useful therapeutic measure. Acknowledgements We wish to thank Dr S. S. Gubbay, Neurologist, Royal Perth Hospital for his help in the care of this patient. The photographs were provided by the Department of Medical Illustrations, Royal Perth Hospital. Accepted for publication: 15 September 1988 References 1 . Lakke JPWF. Classification of extrapyramidal disorders. J Neurol Sci 1981; 51: 311-27. 2. Fahn S. Torsion dystonia: clinical spectrum and treatment. Sem Neurol 1982; 2: 316-22. 3 . Roos RAC, Bruyn GW. Symptomatic dystonias. In: Vinken PJ, Bruyn G W , Klawans H L (eds). Handbook of clinical neurology, Vol. 49, Ch. 32. Amsterdam: Elsevier Science Publishers, 1986. 4. Pettigrew LC, Jankovic J . Hemidystonia: a report of 22 patients and a review of the literature. J Neurol Neurosurg & Psychiatry 1985; 48: 650-7. 5 . Dooling EC, A d a m RD. The pathological anatomy of post hemiplegic athetosis. Brain 1975; 98: 29-48. 6. Grimes JD, Hassen MN, Quarrington AM, D’Alton J . Delayed onset posthemiplegic dystonia: CT demonstration of basal ganglia pathology. Neurology 1982; 32: 1033-5. 7 . Demierre B, Rondot P . Dystonia caused by putaminocapsulo-caudate vascular lesions. J Neurol Neurosurg & Psychiatry 1983; 46: 404-9. 8. Burton K, Farrell K, Li D, Calne DB. Lesions of the putamen and dystonia: CT and magnetic resonance imaging. Neurology 1984; 34: 962-5. 9. Marsden C D , Obeso J A , Zarranz J J , Lang AE. The anatomical basis of symptomatic hemidystonia. Brain 1985; 108: 6 3 - 8 3 . 10. Russo LS. Focal dystonia and lacunar infarction of the basal ganglia: a case report. Arch Neurol 1983; 40: 61-2. 1 1 . Fross RD, Martin WRW, Li D, et al. Lesions of the putamen: their relevance t o dystonia. Neurology 1987; 37: 1 125-9. 12. Burke RD, Fahn S, Gold AP. Delayed-onset dystonia in patients with ‘static’ encephalopathy. J Neurol Neurosurg & Psychiatry 1980; 43: 789-97. BOOK REVIEW FIBREOPTIC BRONCHOSCOPY IN DIAGNOSIS AN D MANAGEMENT. R . M . du Bois, S. W . Clarke. Harper, Row, Artarmon NSW, 1988. 626 pp. Recommended retail price: $140. ISBN: 0 77445513. his book provides a comprehensive review of the techniques and applications of fibreoptic bronchoscopy . It is clearly written and extremely well illustrated by excellent reproductions of chest x-rays, bronchoscopic findings, and histology. Schematic line drawings are used effectively to demonstrate important bronchoscopic and histologic features. Each chapter starts with a general introduction and concludes with a well selected group of case studies illustrating the application of various bronchoscopic techniques. Each chapter has a modest number of references up to 1986. Whilst the book is somewhat expensive, it will almost certainly find a place on the bookshelves of units performing fibreoptic bronchoscopy. It is essential reading for all trainees learning this technique. T C. Mitchell, Associate Professor of Medicine, The University of Queensland. 150 Aust NZ J Med 1989; 19 HANKEY AND SADKA