The Montreal Cognitive Assessment as a screening tool for cognitive dysfunction in Huntington's disease

Cognitive dysfunction is one of the hallmarks of Huntington's disease (HD) and may precede the onset of motor symptoms. The Montreal Cognitive Assessment (MoCA), a brief cognitive screening instrument with high specificity and sensitivity for detecting early cognitive impairments, has not been studied in the HD population. In this study, we compare the MoCA with the mini‐mental state examination (MMSE) as a screening tool for cognitive dysfunction among 53 patients with HD. The mean MMSE score was 26 ± 2.4, and mean MoCA score was 21 ± 4.4. Twenty‐one patients (81%) of those who scored ≥26 on the MMSE had the MoCA score <26. Thirty‐two patients (78%) of those who scored ≥24 on the MMSE had the MoCA score <24. The MoCA may be a more sensitive screening tool for cognitive impairments in HD relative to the MMSE. © 2010 Movement Disorder Society     

Presentation, clinical course, and outcome of childhood stroke.

We reviewed the presentations, clinical courses, and outcomes of 42 children with unilateral hemispheric stroke. Infants with strokes identified within the first few days of life usually presented with seizures. These infants had few abnormal neurologic findings as neonates, but hemiparesis became evident as gross motor development proceeded. Infants with strokes identified later in the first year of life usually presented with pathologic early hand preference without a history of an ictus. During subsequent development, the motor deficits in these children became more evident, producing an apparent progression of the neurologic abnormalities. Strokes identified in older children typically presented as sudden hemiparesis, often associated with seizures. The hemiparesis in these children was most severe at the onset, followed by some improvement in strength in all patients. Functional outcome was variable. At last follow-up, all children were ambulatory, some with clinically apparent hemiparesis. Eight of the 42 children (19%) developed recurrent seizures with an onset ranging from 4 months to more than 10 years (median: 26 months) after the stroke.     

Clinical-computed tomographic correlations of lacunar infarction in the Stroke Data Bank

Lacunar stroke was diagnosed in 337 (26%) of the 1,273 patients with cerebral infarction among the 1,805 total in the Stroke Data Bank. We analyzed the 316 patients with classic lacunar syndromes. Among these, 181 (57%) had pure motor hemiparesis, 63 (20%) sensorimotor syndrome, 33 (10%) ataxic hemiparesis, 21 (7%) pure sensory syndrome, and 18 (6%) dysarthria-clumsy hand syndrome. No striking differences were found among the risk factors for the lacunar subtypes, but differences were found between lacunar stroke as a group and other types of infarcts. Compared to 113 patients with large-vessel atherosclerotic infarction, those with lacunar stroke had fewer previous transient ischemic attacks and strokes. Compared to 246 with cardioembolic infarction, patients with lacunar stroke more frequently had hypertension and diabetes and less frequently had cardiac disease. We found a lesion in 35% of the lacunar stroke patients' computed tomograms, with most lesions located in the internal capsule and corona radiata. The mean infarct volume was greater in patients with pure motor hemiparesis or sensorimotor syndrome than in those with the other lacunar stroke subtypes. In patients with pure motor hemiparesis and infarcts in the posterior limb of the internal capsule, there was a correlation between lesion volume and hemiparesis severity except for the few whose infarct involved the lowest portion of the internal capsule; in these patients severe deficits occurred regardless of lesion volume. Taken together, the computed tomographic correlations with the syndromes of hemiparesis showed only slight support for the classical view of a homunculus in the internal capsule.     

Hemiparkinsonism-hemiatrophy syndrome: clinical and neuroradiologic features

We evaluated 11 patients with hemiparkinson-hemiatrophy syndrome, 6 with body and contralateral cerebral hemispheric hemiatrophy, 4 with only body hemiatrophy, and 1 with just brain hemiatrophy. The mean age of symptom onset was 38.1 years (range, 18 to 54) with 5.2 +/- 3.1 (mean +/- SD) years of illness until the last follow-up visit. The presenting symptom was unilateral tremor in 6 patients, hand dystonia in 2, bradykinesia in 2, and abnormal gait in 1 patient. Three patients had a good response to levodopa, 4 had moderate response, and 2 patients had a poor response. During a mean follow-up period of 1.7 years (range, 4 months to 5 years), the Hoehn and Yahr score changed in only 3 patients: 2 gained 1.5 points and 1 gained 3 points over 2.5 years. We discuss the association between hemiparkinsonism-body hemiatrophy and contralateral hemispheric hemiatrophy, and raise the possibility of early childhood brain insult with delayed-onset parkinsonism.     

Hemiparkinsonism with hemiatrophy

We studied fifteen patients with hemiparkinsonism and ipsilateral hemiatrophy (HP/HA) to better characterize the clinical features of this syndrome and its rate of progression. Patients were distinguished by highly asymmetric parkinsonism with predominant signs on the side of HA, early age of onset (43.7 years versus 60.2 years in our control population of idiopathic Parkinson's disease [IPD], abnormal birth history (7/15), and dystonia occurring prior to levodopa therapy (10/15). In six patients, the mean duration of disease until the initiation of levodopa therapy was 14.2 years, as compared with 4.1 years in our control population of IPD. The slow progression of disease underscores the relatively favorable prognostic significance of HP/HA and its distinction from IPD.     

Neuropsychological abnormalities associated with lacunar infarction

The objective of this study was to assess neuropsychological abnormalities in 40 patients with lacunar infarction. Topography of infarction, presence of isolated or multiple silent infarcts and white matter hyperintensities were correlated with results of neuropsychological tests and subtypes of lacunar infarction. Patients were studied within 1 month after stroke. A total of 21 patients were males and the mean age was 70.7 years; 30% had a single infarction (mean number of infarctions was 3.4). Twelve patients had pure motor hemiparesis, 9 pure sensory stroke, 8 dysarthria-clumsy hand/ataxic hemiparesis, 8 atypical lacunar syndrome, and 3 sensorimotor stroke. The mean score of the Mini-Mental State Examination was 28.4. Mild cognitive impairment of subcortical vascular features occurred in 23 patients and isolated executive disturbances in 4. Neuropsychological results showed that patients with atypical lacunar syndrome followed by pure motor hemiparesis showed significantly more cognitive executive disturbances. Patients with dysarthria-clumsy hand/ataxic hemiparesis accounted for the best scores in some tests of visuoconstructive function and visual memory. In summary, mild neuropsychological disturbances (57.5%) are not infrequent in acute lacunar infarcts especially in patients with atypical lacunar syndrome and pure motor hemiparesis. Neuropsychological impairment should be considered as common clinical feature in acute lacunar infarction.     

Frequency, clinical features and risk factors of lacunar infarction (data from a stroke registry in South India)

Analysis of 893 patients of ischaemic stroke in the stroke registry of Nizam's institute of Medical Sciences, Hyderabad is presented. 16% of them had lacunar infarction. The mean age at presentation was 56.9 years and male to female ratio was 3.5:1. The common risk factors included hypertension(62%),diabetes(38%) and smoking(28%). Six percent had an underlying cardiac source of embolism and none had significant (>50%) extracranial carotid atherosclerosis. In 22% of patients, no obvious risk factors could be identified. The frequency of risk factors was similar in patients with lacunar and non- lacunar infarctions. However, patients with lacunar infarction had higher frequency of diabetes and absence of significant (>50%) extracranial carotid artery disease. Pure motor hemiparesis was the presenting syndrome in 45% patients. Ataxic hemiparesis and sensorimotor stroke accounted for 18% each and dysarthria-clumsy hand syndrome for 14%. This study suggests that the frequency, risk factors and clinical profile of lacunar infarction in our stroke registry is similar to most of the western stroke registries.     

Lacunar syndromes due to intracerebral hemorrhage]

Nine cases (seven men and two women, mean age 64.5 years) of classical lacunar syndromes due to intracerebral hemorrhage are reported. Three patients presented with pure motor hemiparesis (two putaminal hematomas with proportional weakness and one cortical hemorrhage with brachio-crural hemiparesis). Four patients presented with sensorimotor stroke due to thalamo-capsular hemorrhage. The last two patients had thalamic hemorrhage causing ataxic hemiparesis or dysarthria-clumsy hand syndrome. Four subjects had arterial hypertension, one was diabetic, and two were treated with anti-vitamin K. Abrupt onset was noted in all instances. Only one patient experienced moderate inaugural headaches. Good recovery occurred in all cases. Lacunar syndromes are a very uncommon presentation of intracerebral bleeding. Hemorrhages are yet the second etiology of such syndromes. Distinguishing hemorrhage from infarction is not clinically possible and needs early unenhanced CT scan.     

Acute isolated capsular stroke. A clinical study of 148 cases

The objectives of the study were to assess differential features between capsular stroke of ischemic and hemorrhagic origin, and to compare capsular strokes with all other (non-capsular) strokes. Data of 148 patients with isolated capsular stroke were collected from a prospective hospital-based stroke registry in which 2000 consecutive acute stroke patients were included. Isolated capsular stroke accounted for 8.4% of strokes included in the registry (8.4% of ischemic strokes and 10.5% of intracerebral hemorrhages). Capsular stroke of hemorrhagic origin (n = 24) was more severe than ischemic capsular stroke (n = 124) as determined by a significantly higher in-hospital mortality, length of stay, and lower number of patients free of functional deficit at discharge. After multivariate analysis, limb weakness, sudden onset, and sensory symptoms were independently associated with capsular hemorrhage, whereas pure motor hemiparesis appeared to be associated with capsular infarction. In summary, one of each 12 patients with acute ischemic stroke and one of each 10 patients with acute intracerebral hemorrhage had an isolated capsular stroke. Lacunar syndrome was the most frequent clinical presentation being more common (particularly pure motor hemiparesis) in ischemic than in hemorrhagic capsular stroke. Capsular hemorrhage and capsular infarction showed identical risk factor profiles suggesting the same underlying vascular pathology for both conditions.     

When is sensorimotor stroke a lacunar syndrome?

Forty five patients with clear sensorium and no neurological deficits other than unilateral motor and sensory impairment underwent computed tomography (CT). Twenty patients had sensorimotor stroke with impairment of all sensory modalities (type 1). Eight had only impairment of nociceptive sensation (type 2) and 15 had only proprioceptive impairment (type 3). Two patients had sensory impairment in one limb only (type 4). Lacunes were found in patients in the first three groups. However, 80% of those who had hemiparesis and incomplete sensory loss were found to have a lacune or normal CT scan whilst only 33% of those with complete motor or sensory impairment had lacunes. It is proposed that sensorimotor stroke as a lacunar syndrome be best restricted to those with only mild to moderate hemiparesis and sensory impairment in both upper and lower limbs. The degree and extent of sensory and motor involvement may vary, however, possibly dependent on whether the thalamo-geniculate, anterior choroidal or lateral lenticulostriate artery is affected.     

老年人卒中后痫样发作现象的调查研究

This prospective study sought to investigate the clinical,radiological and electroencephalographic(EEG) characteristics of seizures in elderly stroke patients,and their outcomes.Over a 2-year study period,158 consecutive elderly patients with stroke were examined and followed up.Of these patients,32(20%) developed seizures,primarily related to stroke,within a follow up period between 5 months and 2 years.Of these 32 cases,20 experienced infarctions,and 12 experienced hemorrhages.Involvement of cortical regions was detected in most of the patients exhibiting seizures.In these patients,44% of the lesions involved cortical areas exclusively or in addition to subcortical areas observed on computed tomography(CT) images.Twenty-five patients(78%) developed early seizures(within 2 weeks after stroke),and half exhibited immediate post-stroke seizures.None of the patients exhibiting early onset seizures developed recurrent seizures or epilepsy,while 57% of late onset seizures(four cases) developed epilepsy.No specific EEG patterns were apparent in those who later developed epilepsy.Overall,early onset seizures after stroke were found to be relatively common,and did not affect outcome.Late onset seizures were less common,but were associated with chronic epilepsy.     

Idiopathic cervical dystonia: clinical characteristics

We reviewed detailed clinical features of 266 patients with idiopathic cervical dystonia, commonly called spasmodic torticollis. Mean age at onset (41 years), female-to-male ratio (1.9:1), clustering of onset between ages 30 and 59 (70%), familial history of dystonia (12%), and remissions (9.8%) were similar to those found in previous studies. In contrast to the single prior large clinical study of this disorder, no predominance of right-handers or significant thyroid disease was found. Pain, which occurred in 75% of patients and contributed to disability score (p less than 0.01), distinguishes this syndrome from all other focal dystonias. Pain was also strongly associated with constant (vs. intermittent) head turning, severity of head turning, and presence of spasm. Eighty-three percent of patients had deviation of the head of greater than 75% of the time when sitting with the head unsupported (constant head deviation at rest). Of the 97% who had head turning, 81% also had head tilting in various combinations. The 23% with hand tremor had an older age at onset (mean, 46 vs. 41 years; p less than 0.05). An earlier age at onset (p less than 0.05) was seen in patients with a family history of dystonia (mean, 36 years), with trauma shortly preceding symptoms (mean, 36 years), with a change in the direction of head turning (mean, 30 years), and with remissions (mean, 33 years). Jerky movements or forced transient spasms of the head occurred in 62% of the patients, and these patients would be the ones for whom the designation "spasmodic torticollis" could logically apply.(ABSTRACT TRUNCATED AT 250 WORDS)     

Etiology and long-term prognosis of unilateral paramedian pontine infarction with progressive symptoms

Progressive pure motor hemiparesis is a common feature in paramedian pontine infarction. To assess the etiology, clinical course and long-term prognosis of this stroke subtype, we identified 26 patients with progressive symptoms in a retrospective study, treated in our hospital between 1993 and 1998. All patients had more than one stroke risk factor, and in 20, basilar branch disease, an atherosclerotic occlusion of basilar perforating arteries, was causative. Most patients developed severe hemiparesis after a mean time of 3 days, nearly 50% showed deterioration in connection with a fall in systolic blood pressure. The patients were followed up for 4-9 years: 4 patients had further strokes, 5 died, and 17 patients were independent, which may be interpreted as a good long-term prognosis.     

Characterizing Sunflower syndrome: a clinical series

Aims. To characterize the clinical phenotype of Sunflower syndrome. Sunflower syndrome is a rare photosensitive epilepsy syndrome characterized by highly stereotyped seizures, photosensitivity, and heliotropism. Methods. We retrospectively reviewed the medical records of patients seen in the Massachusetts General Hospital for Children (MGHfC) pediatric epilepsy program with a history of Sunflower syndrome. Results. Twenty‐four patients were identified; 18 were female. At the time of initial MGHfC evaluation, patients’ ages ranged from 6.4 to 25 years, with a median age of 11.5 years. All patients presented with hand‐waving episodes (HWEs), although one patient no longer demonstrates this, but now has eye blinking episodes on exposure to light. Four have associated eye fluttering as a component of their most prevalent light‐induced seizures. The average age at onset of HWEs was six years. Seventeen developed other symptoms prior to the onset of HWEs. The most prevalent symptom was an attraction to light and possible absence seizures. Light‐induced seizures were generally refractory to broad‐spectrum antiepileptic drugs (AEDs). Only three patients had a reduction of HWEs with the use of AEDs. Several non‐pharmacological strategies reduced seizure frequency, however, efficacy varied. These non‐pharmacological strategies included avoiding stimulus, focusing on other tasks, and occupying or restraining the hand that was involved in hand‐waving. The use of tinted glasses reduced seizure frequency in 17 patients, however, no patient achieved seizure freedom. Twenty‐two patients had available EEGs, 20 of which showed interictal epileptiform discharges. Additionally, many of the patients experienced a negative impact on their self‐concept due to anxiety, depression, or negative interactions with peers. Conclusion. Sunflower syndrome is a generalized, pharmacoresistant epilepsy with childhood onset and remains poorly understood. To improve clinical care and scientific understanding, long‐term prospective research exploring the natural history, etiology, and effective treatments for Sunflower syndrome should be conducted. [Published with video sequence].     

Progressive supranuclear palsy and a multi-infarct state

In 58 patients with progressive supranuclear palsy (PSP), 19 (32.8%) had CT, MRI, or autopsy evidence of a multi-infarct (MI) state. The clinical findings in the infarct syndrome were similar to idiopathic PSP. Five MI-PSP patients had had a stroke, four had focal dystonia, two had hemiparesis, and one had an intention tremor of recent onset. In contrast, only 5.9% (12.9% of those with CT or MRI) of 426 Parkinson's disease patients had evidence of strokes. One case of PSP studied pathologically was attributed to cerebral amyloid angiopathy.     

The clinico-radiological spectrum of Dyke-Davidoff-Masson syndrome in adults

Dyke-Davidoff-Masson syndrome (DDMS) is characterized by cerebral hemiatrophy, seizure, contralateral hemiplegia/hemiparesis, and mental retardation. In this study, clinical and radiological investigations of seven patients who were diagnosed with DDMS as adult age were evaluated and discussed. Seven patients (four male, three female) were included. The mean age ± SD of the patients was 46 ± 21 years. Clinical presentation of six patients was epileptic seizure. One patient was presented with head trauma due to a fall. Two patients had complex partial seizures, three patients had generalized tonic-clonic seizures (GTC), and one had GTC and myoclonic seizure. Mental retardation was in five patients. A congenital cause was detected in one patient in the etiologic investigation and acquired causes in two patients. In four patients, the etiology was not identified. We observed left-hemisphere involvement in four patients and right-hemisphere involvement in three patients. Brain imaging was performed by CT only in four patients and by MRI only in three patients. All patients were diagnosed with DDMS at adulthood. Atrophy in basal ganglia was detected in five patients, and atrophy in brain stem in four patients. Calvarial thickening was observed in four patients. Three patients had hyperpneumatization in mastoid cells. Sinus hyperpneumatization, including the paranasal and frontal sinuses, was seen in six patients. DDMS can also be diagnosed in adulthood symptomatically (mild–severe) or asymptomatically in adulthood. As a result, DDMS is a syndrome with wide clinical and radiological spectra that can be variably symptomatic at different stages of life.     

Thrombolysis as a factor associated with favorable outcomes in patients with unclear‐onset stroke

Background and purpose: Clinical and radiological features of patients with unclear‐onset stroke do not differ significantly from those with known‐onset stroke. There is a lack of evidence for the safety and efficacy of thrombolysis in patients with unclear‐onset stroke. We sought to provide supportive data on the safety and efficiency of thrombolysis in patients with unclear‐onset stroke. Methods: We retrospectively identified patients with unclear‐onset stroke (<3 h of first found abnormal time) from our stroke registry. We performed following protocols for thrombolysis in patients with unclear‐onset stroke; initial conventional CT‐based intravenous thrombolysis (IVT), repeat MRI during IVT, and then decision to maintain IVT or to perform combined intra‐arterial thrombolysis. In addition, we compared clinical outcomes and safety between thrombolyzed and non‐thrombolyzed patients. Results: A total of 78 patients with unclear‐onset stroke were included. Twenty‐nine patients underwent thrombolysis. Thrombolysis (OR, 6.842; 95% CI, 1.950–24.004; P = 0.003) and baseline NIHSS (OR, 0.769; 95% CI, 0.645–0.917; P = 0.003) were associated with favorable outcomes at 3 months in multivariate logistic regression analysis. The frequency of hemorrhagic transformation and symptomatic ICH was not significantly different between the thrombolyzed and non‐thrombolyzed patients (34.4% vs. 40.7% and 10.3% vs. 8.2%, respectively). Conclusion: The results of this study suggest that thrombolysis in unclear‐onset stroke could be independently associated with favorable outcomes at 3 months and that thrombolysis based on repeat imaging appears to be safely applied to patients with unclear‐onset stroke.     

Pathogenesis of ipsilateral hemiparesis in patients with lateral medullary syndrome

Lateral medullary syndrome is known to cause hemiparesis ipsilateral to the medullary lesion. However, it's clinical significance has not been fully evaluated. In this study, we made clinical and angiographic studies in patients with syndrome to elucidate the pathogenesis of ipsilateral hemiparesis. Thirty-four patients with cerebral infarction presenting with this syndrome were studied. Their mean age was 51 years and all the patients were examined within 6 months of their first attack. Diagnosis of ipsilateral hemiparesis was made if subjective feeling of weakness in the upper and lower extremities was associated with increased deep tendon reflexes. Ipsilateral hemiparesis was observed in 38% of all the patients. Cerebral angiography was performed in 26 patients and divided into two groups; group A with ipsilateral hemiparesis (n = 9) and group B without ipsilateral hemiparesis (n = 7). In 56% of patients in group A, angiography showed non-visualization of ipsilateral vertebral artery (VA) and posterior inferior cerebellar artery (PICA). Non-visualization of these two arteries never occurred in the patients of group B. On the other hand, non-visualization of VA alone was observed in 35% of the patients in group B, but it was none in the patients of group A. However, there were no differences in concerning the frequency of non-visualization of PICA alone or non-occlusion at all between both groups. Therefore, the focal ischemia in the region below the pyramidal decussation due to the occlusion of both VA and PICA, regardless of thrombotic or embolic episode, was considered to be responsible for ipsilateral hemiparesis.(ABSTRACT TRUNCATED AT 250 WORDS)     

Contraversive pushing in non-stroke patients

Abstract Background Pusher syndrome is a disorder of postural control observed in patients with right or left brain damage associated with hemiparesis. Those patients show a peculiar behavior of actively pushing away from the nonhemiparetic side and resisting against passive correction, with a tendency to fall toward the paralyzed side. Thus far this phenomenon has been exclusively associated with stroke patients. Objective We investigate the occurrence, imaging features and clinical evolution of pusher behavior in patients with acute encephalic lesions at a tertiary emergency hospital. Methods Pusher patients were identified from 530 inpatients during a 1 year period. Patients were evaluated using a standardized Scale for Contraversive Pushing (SCP), neurological examination, assessment of neuropsychological symptoms, activities of daily living function and neuroimaging studies. Results We found eight patients (1.5%) with severe contraversive pushing, three female and five male. Age at symptoms onset ranged from 48 to 80 years (mean 65.4). All patients had scores equal or above 1.5 in each tested parameter of the SCP. Six patients (75 %) had right-hemisphere brain damage. A stroke etiology was found in four patients. The other four patients had non-stroke etiology (three traumatic, one metastatic tumor). Stroke patients showed complete recovery of pusher behavior at a mean duration of 15.3 weeks. In patients with brain trauma, pushing behavior was completely resolved in a mean time of 5 weeks. Conclusions The results demonstrate that contraversive pushing may also occur in patients with non-stroke neurological lesions and suggest that resolution of symptoms may vary according to the underlying etiology.