Recurrent unipolar depression with 48-hour cycle and the state--dependent abnormal involuntary movements following right thalamic hemorrhage]

A 70-year-old woman was admitted because of depression and abnormal involuntary movements of her left extremities. Six months before the admission, she developed left hemiparesis caused by right thalamic hemorrhage. On neurological examination, she had mild motor and sensory hemiparesis on the left side. She showed recurrent depression each other days with a 48-hour cycle, and hemichorea-hemiballism appeared in her left upper and lower extremities exclusively on the days with depressive phase. Her depressive symptoms were characterized by psychomotor retardation but not by feeling of suicide or guilty. She was then diagnosed as having a rapid cycler following the right thalamic hemorrhage. To our knowledge, it is extremely rare to see the state-dependent involuntary movements in patients with rapid cycler. The coexistence of post-stroke depression and hemichorea-hemiballism in the present patient may suggest that both disorders appear on the basis of the common pathophysiological mechanism such as dysfunction on frontal cortico-basal ganglia-thalamo-cortical loops.     

Cerebral arteriovenous malformations and movement disorders.

A series of six patients with movement disorders associated with cerebral arteriovenous malformations (AVM) is reported. The AVMs were classified according to the Spetzler-Martin classification as grade V (one patient), grade IV (four patients), and as grade III (one patient). One patient had action-induced hemidystonia caused by a contralateral frontoparietal AVM which compressed the putamen and was supplied partially by enlarged lenticulostriate arteries. Two patients presented with unilateral cortical tremor associated with contralateral high-frontal cortical/subcortical AVMs sparing the basal ganglia. Another patient developed hemidystonia and hemichorea-hemiballism after bleeding of a contralateral temporooccipital AVM and subsequent ischemia. Two patients had focal dystonia after thalamic and basal ganglia hemorrhage from AVMs. Five patients were operated on. The movement disorder was abolished in one patient postoperatively. Different mechanisms were identified that are relevant for the development of AVM-related movement disorders: mass effect, diaschisis, local parenchymal altered cerebral blood flow, and hemorrhagic or ischemic structural lesions.     

Pure Cortical Stroke Causing Hemichorea-Hemiballismus

Background: Movement disorders including hemichorea-hemiballism as the initial presentation of an acute ischemic stroke are uncommon. Structures outside of the deep subcortical areas such as the subthalamic nucleus or basal ganglia are rarely involved. Case Report: We report a case of a 72-year-old man with vascular risk factors who presented with acute onset right-sided hemichorea-hemiballism. Metabolic-, infectious-, and toxic-related conditions were ruled out, his EEG was without epileptiform changes. An MRI confirmed an acute ischemic stroke in the parieto-occipital region without any subcortical structures involved. Atrial Fibrillation was later discovered during his hospitalization and was treated appropriately. Conclusions: Although rare, strokes outside of the subthalamic nucleus can result in hemichorea-hemiballism.     

Hemichorea-hemiballism associated with an ipsilateral intraventricular cyst after resection of a meningioma

We report a patient who developed hemichorea-hemiballism ipsilateral to an intraventricular cyst after resection of a meningioma of the lateral ventricle while under medication with phenytoin. Magnetic resonance imaging demonstrated distortion of the upper brainstem and diencephalon compatible with functional involvement of the contralateral subthalamic nucleus. The movement disorder disappeared after drainage of the cyst. Hemichorea and hemiballism have been reported to be associated with ipsilateral cerebral lesions in rare cases. Review of the literature raises some doubt about the validity of assumptions drawn from previous reports. There is little evidence that hemichorea-hemiballism may be caused by an isolated ipsilateral lesion.     

Hemichorea-hemiballismus and toxoplasmosis in AIDS]

The authors report two cases of hemichorea-hemiballism revealing a toxoplasmic abscess in the subthalamic nucleus during AIDS. Despite the great frequency of this opportunistic infection such cases are exceptional. This rarity seems to be explained by the frequent association of other lesions of the basal ganglia which may prevent the movement disorders. Nevertheless an hemichorea-hemiballism in young people must suggest a cerebral toxoplasmosis. Finally a symptomatic treatment is often necessary; in these cases the use of sodium valproate must be considered, this drug being able to induce a clinical improvement.     

Tremor and dystonia after penetrating diencephalic-mesencephalic trauma

Isolated post-traumatic basal ganglia or brainstem lesions secondary to direct injury resulting in movement disorders, such as tremor or dystonia, are extremely rare. We report an unusual case with a 4-5 Hz rest and postural tremor and focal action-induced dystonia of the left arm. The movement disorder developed within 2 years of the patient sustaining a right-sided mesencephalic-diencephalic lesion due to a penetrating thrust injury with a screwdriver. Magnetic resonance imaging demonstrated involvement of the cerebral peduncle, the substantia nigra, the subthalamic region and the thalamus.     

Lacunar stroke attributable to radiation-induced intracranial arteriopathy

We report the rare presentation of lacunar stroke syndrome secondary to single perforator mouth occlusion from radiation-induced middle cerebral artery (MCA) stem arteriopathy. A 30-year-old female had acute-onset right-sided ataxic hemiparesis and dysarthria. As a child, she had a medulloblastoma of the posterior fossa and had surgery followed by cranial radiotherapy. She had no significant vascular risk factors. Acute CT showed extensive bilateral basal ganglia and left thalamic calcification; DWI showed a left internal capsule lacunar infarct; and MRA and CTA showed a 50% stenosis of the proximal left MCA.     

Non-persistent "doll phenomenon" in a patient with right thalamic infarction]

An 81-year-old right-handed woman was admitted because of acute dysarthria and left hemiparesis. She had lived herself without aids until the admission. On neurological examination she was confused and disoriented. She was ambulant, but had mild dysarthria and mild left hemiparesis. Neuropsychological tests showed severe impairment of memory, mild impairment of visual cognition, decreased fluency of word recall and mild paramnesia, but no acalculia, agraphia, aphasia or apraxia. MRI of the brain showed small infarction in the right anterior thalamus. 123I-IMP SPECT demonstrated a decrease in CBF of the thalamus, basal ganglia and frontal lobe on the right. During admission, she always played with a doll as if she took it as a real baby. This peculiar symptom. "doll phenomenon" continued for approximately three months later. The "doll phenomenon" usually appears in demented patients with diffuse mental deterioration or dysfunction of the frontal lobe. The present patient had not been demented until the onset of the thalamic infarction, and disturbance of cognition caused by the right thalamic infarction probably produced the "doll phenomenon".     

Acute zonal occult outer retinopathy and multiple sclerosis

The case of a 30-year-old woman who had two episodes of photopsia along with sudden-onset monocular visual field defects, developing into bilateral tunnel vision within 4 years, is reported. She also had episodes of a right hemiparesis and right-sided hypoaesthesia, accompanied by severe fatigue. This patient fulfilled the criteria for both clinically definite multiple sclerosis and acute zonal occult outer retinopathy (AZOOR). AZOOR can have an onset with monocular visual field loss, and can be distinguished from optic neuritis. In addition, some observations suggest common neuropathological and inflammatory mechanisms between multiple sclerosis and AZOOR.     

Subthalamic nucleus lesions induce deficits as well as benefits in the hemiparkinsonian rat.

Lesions of the subthalamic nucleus can restore some imbalances in motor output of the basal ganglia induced by nigrostriatal dopamine depletion, and have been proposed as a potential therapy for Parkinson's disease. Although there is substantial supporting evidence from experimental studies in both rats and primates, there is less information on the effects of subthalamic lesions alone. In order to characterize potential side effects, the present study evaluates the behavioural effects of unilateral excitotoxic lesions of the subthalamic nucleus in rats that have previously received either unilateral saline or 6-hydroxydopamine injections into the nigrostriatal bundle on the same side. The 6-hydroxydopamine lesions induced ipsilateral orientation asymmetries in head position and body axis bias, rotational asymmetries following injections of direct or indirect dopamine agonists, neglect of contralateral stimuli, and a reduction in the numbers of pellets retrieved with the contralateral paw in a skilled reaching task. Subsequent excitotoxic lesions of the subthalamic nucleus reduced (but did not abolish) rotational asymmetries, had no effects on the measures of neglect and skilled paw-reaching, and produced contralateral orientation biases in head turning and body axis curling. Rats that received subthalamic lesions alone exhibited de novo impairments comprising contralateral biases in the orientation tests. These results support a neuromodulatory role of the subthalamic nucleus in regulating motor outputs of the basal ganglia, and caution that there may be distinct side effects of the lesion by itself. Whereas some impairments attributable to dopamine depletion may be alleviated by subthalamic manipulations, other symptoms are not, or may even be aggravated.     

Primary CNS lymphoma presenting as a choreic movement disorder followed by segmental dystonia

Clinical presentation of primary CNS lymphoma with an extrapyramidal movement disorder has not been recorded. A 66-year-old woman presented with chorea involving her left arm and subsequently developed right-sided segmental dystonia with prominent hemifacial dystonic spasms, milder torticollis and dystonia of the right arm. Investigations revealed primary CNS lymphoma with extensive involvement of the right-sided basal ganglia as well as lesions confined to the head of the left caudate nucleus and the corpus callosum. Chorea of her left arm subsided with progressing disease while remission of right-sided segmental dystonia was observed following radiotherapy of the brain. This patient's findings and a review of the literature suggest a possible relation between cranio-cervical dystonia and pathology affecting the head of the caudate nucleus.     

Dystonia and tremor secondary to a pediatric thalamic stroke

A previously healthy 10-year-old girl developed a right hemiparesis with sensory loss secondary to a posterolateral thalamic infarct. Despite improvement in strength, three weeks later a 4 Hz kinetic tremor appeared in the right hand accompanied by dystonia in the right upper and lower limbs. Basal ganglia vascular lesions are rare in childhood and movement disorders secondary to such lesions even more so. A thorough work-up failed to disclose the etiology. Our patient illustrates that dystonia and tremor secondary to posterolateral thalamic infarctions are also apt to occur in children and, unlike the adult picture, abnormal movements may develop very soon after the insult.     

Improvement in hemiballism after transient hypoxia in a case of subthalamic hemorrhage

A 73-year-old man was admitted complaining of violent involuntary movement in the left upper and lower extremities. He had a ten-year history of hypertension and had had a left thalamic hemorrhage 6 years before admission. On neurological examination Horner's sign in the right eye, typical hemiballism in the left extremities and right hemiparesis, which was caused by the previous left thalamic hemorrhage, were observed. CT scan and MRI revealed recent hemorrhage in the right subthalamic nucleus. Haloperidol, tiapride and diazepam were administered to ameliorate the ballism, but they had to be reduced in amount because of the development of parkinsonism. Two months after onset, when there was still moderate ballism, he suffocated due to a swallowing disturbance. After two hours' coma, consciousness returned gradually. Twenty-four hours after suffocation, the neurological examination revealed normal consciousness and no deterioration in other neurological symptoms, but the ballism had almost disappeared without medication. No change was detected in MRI findings and the blood flows in the basal ganglia before and after suffocation. It is interesting that transient hypoxia due to suffocation reduced hemiballism in this patient without neuroradiological findings of ischemia in the basal ganglia. The mechanisms of reduction of hemiballism after transient hypoxia were discussed.     

A case of Foix-Chavany-Marie syndrome and crossed aphasia after right corona radiata infarction with history of left hemispheric infarction]

Foix-Chavany-Marie syndrome (FCMS) is a syndrome that presents facio-pharyngo-glosso-masticatory diplegia with automatic voluntary dissociation. Its most common etiology is stroke in the regions of bilateral opercula. We described a 75-year-old woman with FCMS and crossed aphasia. She had cerebral infarction of left middle cerebral artery territory 23 years before. At that time she had transient right hemiparesis, but no aphasia. This time, she suddenly became mute and was brought to our hospital. Neurological examination revealed severe weakness in her bilateral lower face, pharynx, tongue, and sternocleidomastoideus. She had no weakness of limbs. Her listening comprehension was moderately disturbed and handwriting was paragraphic. Her emotional facial movement was maintained despite of disturbed volitional facial movement. CT scan disclosed fresh infarction at the right corona radiata and old infarction at the left middle cerebral artery territory. In this patient, lesions at the left operculum and right corona radiata with the preserved right operculum gave rise to FCMS. This implies following possibilities: 1) the corticobulbar tract and corticospinal tract run separately at the corona radiata, 2) volitional and emotional tracts of facial movement run separately at the corona radiata. It was demonstrated that FCMS is not always caused by bilateral operculum lesions. Our patient did not show aphasia after the first stroke including left language area, but became severely aphasic after the right corona radiata infarction. Simultaneous occurrence of FCMS and aphasia after corona radiata lesion suggested that the corticobulbar tract and a tract that conducts linguistic information are running adjacently in the corona radiata. Our case suggested that restricted corona radiata lesion may cause severe subcortical aphasia and in case of additional contralateral corticobulbar tract lesion, severe dysarthria may occur.     

Ipsilateral hemiparesis caused by a corona radiata infarct after a previous stroke on the opposite side

Ipsilateral hemiparesis after a supratentorial stroke is rare. However, the role of the reorganization of the unaffected hemisphere in recovery after a stroke is poorly understood. Two patients developed ipsilateral hemiparesis after a left corona radiata infarct. Both of these patients had previously experienced contralateral hemiparesis after a right-sided supratentorial stroke. Functional magnetic resonance imaging demonstrated bilateral motor area activation during paretic left hand movement. This finding suggests that the ipsilateral hemiparesis was caused by a new stroke in the ipsilateral motor system that was functionally reorganized after the previous stroke.     

Monocular elevation paresis caused by an ipsilateral lesion

A 52-year-old woman presented with a paresis of elevation of the right eye, equally severe in abduction and adduction (monocular elevation paresis). CT demonstrated a small, right-sided tumor of the mesodiencephalic junction. Monocular elevation paresis has been attributed to lesions of the contralateral pretectum, although proof has been lacking. Now, with documentation of an ipsilateral brainstem etiology, we can postulate a lesion affecting the upgaze efferents from the ipsilateral rostral interstitial nucleus of the medial longitudinal fasciculus.     

Automatisms with preserved responsiveness and ictal aphasia: contradictory lateralising signs during a dominant temporal lobe seizure.

The 25-year-old right-handed woman suffering from temporal lobe epilepsy (TLE) was referred to our centre for presurgical evaluation. MRI showed a right-sided hippocampal sclerosis. During video-EEG-recorded seizures, abdominal aura was followed by oral automatisms, during which she was completely reactive to external stimuli, although she was unable to speak. Ictal EEG showed right temporal seizure pattern, without contralateral propagation. She had abnormal speech postictally. Speech-activated functional transcranial Doppler sonography revealed right-sided speech dominance. She has become seizure free after a right-sided amygdalo-hippocampectomy. In our patient, contradictory clinical ictal lateralising signs (automatisms with preserved responsiveness vs. ictal and postictal dysphasia) occurred during right-, speech-dominant-sided seizures. This is the first report when automatisms with preserved consciousness occurred during a seizure originating and involving the speech-dominant hemisphere.     

Ipsilateral putamen hyperintensity on T1-weighted MRI in non-ketotic hyperglycemia with hemiballism-hemichorea: a case report

Movement disorders are often caused by lesions in the contralateral basal ganglia. This report presents an elderly woman with hemiballism–hemichorea induced by non-ketotic hyperglycemia, whose brain CT revealed a hyperdense lesion over the ipsilateral putamen. Concomitantly, MR image revealed a putaminal hyperintensity on T1-weighted image and hypointensity on T2-weighted image. A follow-up brain CT 4 months after remission of the hyperkinesia revealed that the abnormal intensity had been completely resolved.     

Stroke: a rare presentation of cardiac hydatidosis

A 13-year-old boy presented with acute stroke leading to right-sided hemiparesis. A contrast CT scan of the brain showed a hemorrhagic infarct in the left basal ganglia region with surrounding edema. Echocardiography showed a hydatid cyst in the right atrial chamber extending into the left atrium. A single hepatic hydatid was also seen.     

Ipsilateral and contralateral subthalamic activity after unilateral dopaminergic lesion

Unilateral lesions of the dopaminergic nigral neurons in rats are currently used as a model of Parkinson's disease. However, several neurochemical studies have questioned the possible influence of the lesioned side on the contralateral non-lesioned side. To address this question, electrophysiological recordings in the ipsilateral and contralateral subthalamic nucleus was performed on anaesthetized rats, 3, 7 and 14 days after induction of a unilateral dopaminergic lesion. At these three times, the mean discharge rate of the subthalamic neurons recorded ipsilateral to the lesion was increased by 85, 176 and 127%, respectively, whereas this rate was decreased by 16, 27 and 43%, respectively, in the opposite subthalamic nucleus. This result emphasizes the importance of interhemispheric regulation of this structure, contrasting with the unilateralized current model of the functional organization of the basal ganglia.