- Email: [email protected]
Hemidystonia due to porencephalic cyst
Hemidystonia due to porencephalic cyst A ease report Serap Stitqii Saygl, Ne~e S u b u t a y O z t e k i n , O k a y Sarlba~, M u z a f f e r E r y l l m a z a n d T u r g u t Zileli*
Introduction Summary Torsion dystonia is characterized by inappropriate prolonged muscle contractions which forcefully distort the body in typical posture 1. Hemi dystonia (HD) is generally due to focal brain lesions 1-11. H D due to porencephalic cyst (PC) was reported in only one patient 3. We present a patient with H D in which the CT scan showed a PC involving the caudate nucleus.
We present a 28-year old patient with left hemidystonia since early childhood after head trauma. This is the first report of a patient who had hemidystonia due to CT proven porencephalic cyst involving the caudate nucleus. Key words: Porencephaly, caudate nucleus, computed tomography, dystonia, hemidystonia
Case report A 28-year old man was referred because of involuntary movements on the left arm and leg. Pregnancy, delivery and development had been normal until he sustained head trauma at the age of two. The detailed history of the head trauma could not be obtained. Involuntary movements began to manifest after the head trauma and worsened over the last 8 years. There was no family history. Examination revealed a dystonic posture of the left arm on action with the wrist going into ulnar deviation and the fingers extending (Fig. 1). The left foot showed plantar flexion and inversion. On the assesment of the motor function there was quite good grip on the left although not as strong as the right. Sensation and tendon reflexes were normal. A C T scan revealed a hypodense lesion in-
Fig. 1: The left arm that was outstreched showed dystonic movements.
* Hacettepe University, Department of Neurology, School of Medicine, Ankara, Turkey. Address for correspondence: Dr. Serap Satca Saygt, Hacettepe (lniversitesi Hastanesi, N6roloji Ann Bilim Dab, 06100 Ankara, Turkey. Accepted 13-6-90 Clin Neurol Neurosurg 1991. Vol. 93-3
231
who developed a focal dystonia of the left arm beginning four years after a non-concussive head injury, associated with calcification of the right caudate nucleus. In a case of post-traumatic H D reported by Brett et al. the points of interest were the delayed onset of dystonia and the finding of a lesion extending in to the putamen from the posterior limb of the internal capsule on CT scan. It was reported as a case of posttraumatic HD with similar lesion by Andrew et al., but HD had occured the day following a head injury. In the last two cases, head trauma was not severe. Pettigrew reported a 2 year old male with right HD due to left PC and left cerebral atrophy but there was no detailed knowledge about localisation and CT findings. We believe that our case is the first report that CT evidence showed PC as a cause of HD and this finding in our patient emphasizes the importance of CT in the diagnosis of underlying of focal pathology in the HD. References 1
Fig. 2: CT with intrathecal contrast material: the lesion involving right caudate nucleus and extending frontal lobe communicates with lateral ventricul.
2
3
volving the right caudate nucleus and extending into the right frontal lobe. When contrast material was given intrathecally, the lesion filled with it (Fig. 2). The patient was treated with baclofen and diazepam with a slight improvement of symptoms. Discussion
Basal ganglia pathology was consistently present in the symptomatic dystonias 1-1t. CT evidence of lesions in the contralateral putamen, caudate nucleus, thalamus had been seen with hemidystonias that follow stroke 17, trauma1-3, 8t~ or tumorl'lL Lesions in all three sites have in common their ability to disrupt the thalamic input to frontal cortex. The lesion of our patient as revealed by CT involves the caudate nucleus and may disrupt frontal cortex input and output. Messimy et al. reported a thirty year old man 232
4
5 6
7
8
9
10
n
MARSDEN CD, OBESO JA, Z A R R A N Z JJ, LANG AE.
The
anatomical basis of symptomatic hemidystonia. Brain, 1985; 108:463-483. ZEMANW, WHITLOCKCr Symptomatic dystonias In: VlNKEN PJ, BRUYNGW (eds): Handbook of Clinical Neurology. Elsevier, North Holland Inc. New York. 1968; pp. 544-566. PETnOREWLC, JA~KOVICJ. Hemidystonia: A report of 22 patients and a review of the literature. J Neurol Neurosurg Psychiatry 1985; 48:650-7. DEMIERREB, RANDOTP. Dystonia caused by putamino capsulo-caudate vascular lesions. J Neurol Neurosurg Psychiatry 1983; 96:404-9. RUSSOLJ. Focal dystonia, lacunar infarction of the basal ganglia: a case report. Arch Neurol, 1983; 40:61-2. BURTON K, FARREL K, L1 CALNE DB. Lesions of the putamen and dystonia: CT and magnetic resonance imaging. Neurology, 1984; 34:962-5. CrIROUDM, DUMASR. Dystonie secondaire aun infarctus putamino-capsulo-caude chez l'enfant. Rev Neurol. (Paris), 1988; 144 (5):375-7. MESSIMY R, D1EBLER C, M E T Z G E R J. Dystonie d e t o r s i o n du membre superior gauche probablement consecutive a un traumatisme cranien. Rev Neurol. (Paris). 1977; 133:199-206.
Progressive hemidystonia due to focal basal ganglia lesion
BRE'I~F EM, HOARE RD, SHEEHY MP, MARSDEN CD.
after mild head trauma. J Neurol Neurosurg Psychiatry. 1981; 44:8461. ANDRES J, FOWLER C, HARRISON MJG. Hemidystonia d u e to focal basal ganglia lesion after head injury and improved by stereotaxic thalamotomy. J Neurol Neurosurg Psychiatry, 1982; 45:276. NARTONA, J, OBESO JA, TURON T. Hemydystonia secondary to localized basal ganglia tumour. J Neurol Neurosurg Psychiatry, 1984; 47:704-9.
Introduction Summary Torsion dystonia is characterized by inappropriate prolonged muscle contractions which forcefully distort the body in typical posture 1. Hemi dystonia (HD) is generally due to focal brain lesions 1-11. H D due to porencephalic cyst (PC) was reported in only one patient 3. We present a patient with H D in which the CT scan showed a PC involving the caudate nucleus.
We present a 28-year old patient with left hemidystonia since early childhood after head trauma. This is the first report of a patient who had hemidystonia due to CT proven porencephalic cyst involving the caudate nucleus. Key words: Porencephaly, caudate nucleus, computed tomography, dystonia, hemidystonia
Case report A 28-year old man was referred because of involuntary movements on the left arm and leg. Pregnancy, delivery and development had been normal until he sustained head trauma at the age of two. The detailed history of the head trauma could not be obtained. Involuntary movements began to manifest after the head trauma and worsened over the last 8 years. There was no family history. Examination revealed a dystonic posture of the left arm on action with the wrist going into ulnar deviation and the fingers extending (Fig. 1). The left foot showed plantar flexion and inversion. On the assesment of the motor function there was quite good grip on the left although not as strong as the right. Sensation and tendon reflexes were normal. A C T scan revealed a hypodense lesion in-
Fig. 1: The left arm that was outstreched showed dystonic movements.
* Hacettepe University, Department of Neurology, School of Medicine, Ankara, Turkey. Address for correspondence: Dr. Serap Satca Saygt, Hacettepe (lniversitesi Hastanesi, N6roloji Ann Bilim Dab, 06100 Ankara, Turkey. Accepted 13-6-90 Clin Neurol Neurosurg 1991. Vol. 93-3
231
who developed a focal dystonia of the left arm beginning four years after a non-concussive head injury, associated with calcification of the right caudate nucleus. In a case of post-traumatic H D reported by Brett et al. the points of interest were the delayed onset of dystonia and the finding of a lesion extending in to the putamen from the posterior limb of the internal capsule on CT scan. It was reported as a case of posttraumatic HD with similar lesion by Andrew et al., but HD had occured the day following a head injury. In the last two cases, head trauma was not severe. Pettigrew reported a 2 year old male with right HD due to left PC and left cerebral atrophy but there was no detailed knowledge about localisation and CT findings. We believe that our case is the first report that CT evidence showed PC as a cause of HD and this finding in our patient emphasizes the importance of CT in the diagnosis of underlying of focal pathology in the HD. References 1
Fig. 2: CT with intrathecal contrast material: the lesion involving right caudate nucleus and extending frontal lobe communicates with lateral ventricul.
2
3
volving the right caudate nucleus and extending into the right frontal lobe. When contrast material was given intrathecally, the lesion filled with it (Fig. 2). The patient was treated with baclofen and diazepam with a slight improvement of symptoms. Discussion
Basal ganglia pathology was consistently present in the symptomatic dystonias 1-1t. CT evidence of lesions in the contralateral putamen, caudate nucleus, thalamus had been seen with hemidystonias that follow stroke 17, trauma1-3, 8t~ or tumorl'lL Lesions in all three sites have in common their ability to disrupt the thalamic input to frontal cortex. The lesion of our patient as revealed by CT involves the caudate nucleus and may disrupt frontal cortex input and output. Messimy et al. reported a thirty year old man 232
4
5 6
7
8
9
10
n
MARSDEN CD, OBESO JA, Z A R R A N Z JJ, LANG AE.
The
anatomical basis of symptomatic hemidystonia. Brain, 1985; 108:463-483. ZEMANW, WHITLOCKCr Symptomatic dystonias In: VlNKEN PJ, BRUYNGW (eds): Handbook of Clinical Neurology. Elsevier, North Holland Inc. New York. 1968; pp. 544-566. PETnOREWLC, JA~KOVICJ. Hemidystonia: A report of 22 patients and a review of the literature. J Neurol Neurosurg Psychiatry 1985; 48:650-7. DEMIERREB, RANDOTP. Dystonia caused by putamino capsulo-caudate vascular lesions. J Neurol Neurosurg Psychiatry 1983; 96:404-9. RUSSOLJ. Focal dystonia, lacunar infarction of the basal ganglia: a case report. Arch Neurol, 1983; 40:61-2. BURTON K, FARREL K, L1 CALNE DB. Lesions of the putamen and dystonia: CT and magnetic resonance imaging. Neurology, 1984; 34:962-5. CrIROUDM, DUMASR. Dystonie secondaire aun infarctus putamino-capsulo-caude chez l'enfant. Rev Neurol. (Paris), 1988; 144 (5):375-7. MESSIMY R, D1EBLER C, M E T Z G E R J. Dystonie d e t o r s i o n du membre superior gauche probablement consecutive a un traumatisme cranien. Rev Neurol. (Paris). 1977; 133:199-206.
Progressive hemidystonia due to focal basal ganglia lesion
BRE'I~F EM, HOARE RD, SHEEHY MP, MARSDEN CD.
after mild head trauma. J Neurol Neurosurg Psychiatry. 1981; 44:8461. ANDRES J, FOWLER C, HARRISON MJG. Hemidystonia d u e to focal basal ganglia lesion after head injury and improved by stereotaxic thalamotomy. J Neurol Neurosurg Psychiatry, 1982; 45:276. NARTONA, J, OBESO JA, TURON T. Hemydystonia secondary to localized basal ganglia tumour. J Neurol Neurosurg Psychiatry, 1984; 47:704-9.