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PubMed1993

The capsular warning syndrome: pathogenesis and clinical features.

Transient ischemic attacks (TIAs) are not homogeneous and may consist of subsets with mechanisms as varied as their stroke counterparts. We describe a form of TIA in 50 patients where crescendo episodes of ischemia were restricted to the region of the internal capsule, usually causing symptoms affecting face, arm, and leg. These patients composed 4.5% of a consecutive series of patients admitted with TIAs over a 15-year period and 33% of all TIAs classified as subcortical. We believe that the ischemia was most often due to hemodynamic phenomena in diseases, single, small penetrating vessels. When cerebral infarction developed, it was usually lacunar and involved a single penetrating vessel, although occasionally striatocapsular or anterior choroidal artery territory infarction occurred. There was no evidence of artery-to-artery or heart-to-artery embolism. Resistance to various forms of therapy, including hemodiluting, anticoagulant, and thrombolytic agents, was common. Because of dramatic and easily recognizable clinical presentation, apparent specific pathophysiologic mechanism, and the development of early capsular stroke in a high proportion of cases (42%), we have termed this the "capsular warning syndrome."

Aged↗
PubMed1994

Micrographia due to focal cerebral lesions as seen in the dysarthria-clumsy hand syndrome.

Five patients with dysarthria-clumsy hand syndrome developed micrographia. Four had lacunar infarctions involving the putamen or the genu of the internal capsule; the fifth patient had a small hemorrhage in the putamen. Micrographia may be a sign of focal cerebral disease, and basal ganglia dysfunction may be involved in the supratentorial dysarthria-clumsy hand syndrome.

Aged↗
PubMed1997

Adult-onset Krabbe disease with homozygous T1853C mutation in the galactocerebrosidase gene. Unusual MRI findings of corticospinal tract demyelination.

A 51-year-old woman developed a slowly progressive spastic paraparesis and diminished vibration sense beginning at age 38. Intellectual capacity was normal. Krabbe disease was confirmed by markedly reduced leukocyte galactocerebrosidase (GALC) activity, typical inclusions in Schwann cell cytoplasm, and an identification of the homozygous point mutation T1835C (Leu618Ser) in the GALC gene. T2-weighted MRI of the brain showed symmetric high-signal-intensity lesions in the bilateral frontoparietal white matter, the centrum semiovale, and the posterior limb of the internal capsule with sparing of the periventricular white matter. This case is unusual because of the late onset, protracted clinical course, and MRI findings of demyelination confined to the corticospinal tracts.

Age of Onset↗
PubMed1998

MRI in juvenile ALS: a patient report.

We describe the MR images of a patient with juvenile ALS. MRI of the brain showed bilateral hyperintensities along the corticospinal tracts extending from the corona radiata to the brainstem on T2-weighted images. These findings should be differentiated from the slight hyperintensities seen in the posterior limbs of the internal capsules in normal subjects.

Adolescent↗
PubMed1998

Wallerian degeneration of the pyramidal tract does not affect stroke rehabilitation outcome.

OBJECTIVE: To test whether Wallerian degeneration (WD) of the pyramidal tract as signaled by MRI affects rehabilitation outcome in patients with subcortical infarction (internal capsule or corona radiata). BACKGROUND: Recent radiologic evidence suggests that WD occurs no earlier than 3 months after a subcortical infarction. METHODS: A total of 77 consecutive patients with pure motor hemiparesis due to an initial subcortical infarction were assessed on admission and discharge with the Functional Independence Measure (FIM) for disability and Stroke Impairment Assessment Set (SIAS, full = 25) for impairment. WD was defined by a high-intensity area detected along the pyramidal tract below the level of lesion on T2-weighted MR image (WD+). RESULTS: Age, sex, side of stroke, Mini-Mental State Examination score, and volume of lesion were comparable for each group. Length of stay (LOS) was significantly longer (p < 0.05) in WD+ (130 days) than in WD- (105 days). There was no difference in the change of FIM (WD+, 99 to 111; WD-, 95 to 107) or SIAS measures (WD+, 12 to 16; WD-, 13 to 16) made on admission and discharge, nor was there any effect of the timing of the rehabilitation experience (< or = 90 days or >90 days after stroke). CONCLUSIONS: After stroke, apparent WD of the pyramidal tract may slow functional recovery but does not limit final rehabilitation outcome of pure motor hemiparesis. Study of the mechanisms of compensation for this delayed pyramidal tract degeneration will enhance the scientific basis for rehabilitation.

Cerebral Infarction↗
PubMed1999

Progressive motor deficits in lacunar infarction.

OBJECTIVE: To study the clinical characteristics of the progression of motor deficits in lacunar stroke patients. BACKGROUND: Some patients with lacunar infarction have progression of their neurologic deficits, but it is not known which patients will progress or why they progress. METHODS: The authors evaluated 92 consecutive patients (47 men, 45 women; age, 69.4 +/- 10.9 years [mean +/- SD]) with first-ever stroke due to supratentorial lacunes in the internal capsule or the corona radiata. By defining lacunar infarction in which motor deficits progressed between admission and the day after admission as progressive lacunar infarction, the authors compared progressive lacunar infarction with stable lacunar infarction. RESULTS: Of 92 patients, 25 (27%) had progression of deficits. Diabetes mellitus (p = 0.02) and severity of motor deficit on admission (p = 0.006) were related independently to progression in a logistic multiple regression analysis. Size of the infarct was slightly larger (1.2 +/- 0.4 cm2 versus 0.9 +/- 0.5 cm2; p = 0.01) and functional status at discharge was worse (median Barthel index, 45 versus 100; p < 0.001) in patients with progressive infarction than in those without progression. There were no significant differences between the two groups regarding the site of the infarct or blood pressure or hematocrit levels on or after admission. CONCLUSIONS: The progression of motor deficits is associated with a relatively poor functional outcome. Diabetes mellitus and the severity of motor deficit on admission may predict progression of motor deficits.

Activities of Daily Living↗
PubMed1999

Leukoencephalopathy and raised brain lactate from heroin vapor inhalation ("chasing the dragon")

BACKGROUND: Inhalation of heated heroin vapor ("chasing the dragon"), which is gaining popularity among drug users seeking to avoid the risks of parenteral drug administration, can produce progressive spongiform leukoencephalopathy. METHODS: We studied the clinical phenotype and course, MRI, MRS, and brain pathology in the first American patients described with this syndrome. RESULTS: Two of the three heroin users studied inhaled heroin pyrolysate together daily over the course of 2 weeks. They developed ataxia, dysmetria, and dysarthria. Patient 1 progressed to an akinetic mute state with decorticate posture and subsequent spastic quadriparesis. Patient 2 developed a mild spastic quadriparesis and gait freezing. Patient 3 was asymptomatic following less heroin exposure. Brain MRI showed diffuse, symmetrical white matter hyperintensities in the cerebellum, posterior cerebrum, posterior limbs of the internal capsule, splenium of the corpus callosum, medial lemniscus, and lateral brainstem. MRS showed elevated lactate. Brain biopsy (Patient 1) showed white matter spongiform degeneration with relative sparing of U-fibers; electron microscopy revealed intramyelinic vacuolation with splitting of intraperiod lines. Progressive deterioration occurred in Patients 1 and 2 over 4 weeks. Both were treated with antioxidants including oral coenzyme Q, and clinical improvement occurred. Patient 1 recovered nearly completely over 24 months. Patient 2 improved, but developed a delayed-onset cerebellar hand tremor. Both still have white matter abnormalities on MRI and MRS. CONCLUSIONS: Elevated lactate in white matter and the possible response to antioxidants suggests mitochondrial dysfunction in progressive spongiform leukoencephalopathy following inhalation of heated heroin vapor.

Administration, Inhalation↗
PubMed2000

Autosomal dominant diffuse leukoencephalopathy with neuroaxonal spheroids.

OBJECTIVE: To provide clinical, MRI, and histopathologic findings in a rare white matter disorder with autosomal dominant inheritance, so-called hereditary diffuse leukoencephalopathy with spheroids (HDLS). BACKGROUND: Progressive leukoencephalopathies often constitute a diagnostic dilemma in both children and adults. In some cases, histopathologic examination of brain tissue is required for a classifying diagnosis. METHODS: Clinical history, MRI, and autopsy findings were reviewed in three patients with HDLS: a father, his daughter, and an unrelated patient. RESULTS: Clinical history consisted of an adult-onset neurologic deterioration with signs of frontal lobe dysfunction, epilepsy, spasticity, ataxia, and mild extrapyramidal disturbances. MRI findings included cerebral atrophy and patchy white matter changes, most pronounced in the frontal and frontoparietal area with extension through the posterior limb of the internal capsule into the pyramidal tracts of the brainstem. Autopsy in two patients revealed a leukoencephalopathy with frontoparietal and frontal preponderance and numerous neuroaxonal spheroids in the abnormal white matter. The pyramidal tracts were affected throughout the brainstem. CONCLUSION: Similar clinical and histopathologic findings have been reported in members of a Swedish pedigree. The homogeneity of the findings strongly suggests that HDLS is a distinct disease entity. In the absence of a biochemical or genetic marker, a definitive diagnosis requires histopathologic confirmation in one of the affected family members. Neuroaxonal spheroids.

Adult↗
PubMed2001

MRI identification of early white matter injury in anoxic-ischemic encephalopathy.

BACKGROUND: Anoxic-ischemic encephalopathy (AIE) affects the gray matter more than the white matter. Recent animal experiments suggest that the white matter is more sensitive to ischemia than previously thought. The authors describe the MRI findings in seven patients with AIE who demonstrate early preferential involvement of the white matter. MATERIALS AND METHODS: A retrospective case series study was performed, including seven patients with AIE who underwent MRI of the brain within 7 days of insult. Demographic information, type of insult, clinical examination findings, EEG findings, and clinical outcome were obtained. MRI studies were reviewed with specific attention to the cortex, deep gray matter, and the white matter structures. Mean apparent diffusion coefficient (ADC) was calculated in regions of interest placed in the cerebellar hemispheres, putamen, thalamus, splenium of corpus callosum, centrum semiovale, and medial frontal cortex. RESULTS: The causes of AIE were cardiac arrhythmias in two patients, myocardial infarction in one, drug overdose in two, carbon monoxide poisoning in one, and respiratory failure and sepsis in one. The median time to MRI was 2.5 days. Symmetric areas of restricted diffusion were found in the periventricular white matter tracts (7/7 patients), the corpus callosum (6/7 patients), internal capsule (5/7 patients), and the subcortical association fibers (3/7 patients). ADC maps confirmed the restricted diffusion. Gray matter involvement was seen in three patients, and was more prominent on conventional imaging sequences compared with diffusion-weighted imaging. A subtle decrease in mean ADC was seen in cortex. CONCLUSIONS: Prominent, symmetric restricted diffusion can occur early after AIE in white matter, whereas gray matter involvement may be less prominent. Further studies involving a larger sample and serial imaging are required to confirm these preliminary findings.

Adult↗
PubMed2001

Volumetric analysis reveals corticospinal tract degeneration and extramotor involvement in ALS.

BACKGROUND: Pathologic changes in the motor cortex and corticospinal tracts in ALS may be reflected by abnormal signal intensities on conventional MRI. The sensitivity of these changes in detecting underlying pathology remains unclear. METHOD: The authors used automated image analysis to quantify volumes of cerebral gray and white matter in 16 patients with ALS (eight limb onset, eight bulbar onset) and eight normal controls. Previously they had demonstrated a reduction in N-acetyl aspartate/creatine + phosphocreatine (NAA/[Cr + PCr]) measured by (1)H-MRS in the subcortical white matter in the motor cortex region in the patients with bulbar-onset ALS. To determine whether this resulted from axonal degeneration, they also compared gray and white matter volumes in the patients with limb- and bulbar-onset ALS. RESULTS: There were no differences in the total brain volumes of gray or white matter for the three subject groups (p > 0.23). Comparison of the total ALS group and controls revealed localized deficits in gray matter volume centered on Brodmann areas 8, 9, and 10 bilaterally. Comparison of the patients with limb- and bulbar-onset ALS revealed deficits in the white matter volume in the bulbar-onset group, extending bilaterally from the precentral gyrus into the internal capsule and brainstem, consistent with the course of the corticospinal tract. There was no loss in gray matter volume in the precentral gyri. CONCLUSIONS: The loss of gray matter in the frontal regions (total ALS group) provides further support that ALS is a multisystem disorder. In addition, there is in vivo evidence of axonal degeneration in the subcortical white matter in the motor region in patients with bulbar-onset ALS. This is consistent with a "dying back" process affecting cortical motoneurons in bulbar-onset ALS.

Adult↗
PubMed2002

Diffusion tensor imaging of periventricular leukomalacia shows affected sensory cortex white matter pathways.

The authors used diffusion-tensor imaging to examine central white matter pathways in two children with spastic quadriplegic cerebral palsy. Corticospinal tracts projecting from cortex to brainstem resembled controls. In contrast, posterior regions of the corpus callosum, internal capsule, and corona radiata were markedly reduced, primarily in white matter fibers connected to sensory cortex. These findings suggest that the motor impairment in periventricular leukomalacia may, in part, reflect disruption of sensory connections outside classic pyramidal motor pathways.

Brain Mapping↗
PubMed2003

Central post-stroke pain or paresthesia in lenticulocapsular hemorrhages.

Twenty patients were studied who developed central poststroke pain or paresthesia after lenticulocapsular hemorrhage. Pain or paresthesia occurred 0 to 24 months after the onset, more prominently in the leg than other body parts. The symptoms were described as numb, cold, burning, aching, swollen, and squeezing in various combinations. The mean score of the visual numerical scale was 5.6. The lesions involved the dorsal part of the posterior limb of the internal capsule, probably damaging the thalamocortical sensory pathway.

Adult↗
PubMed2006

Diffusion tensor imaging of white matter after cranial radiation in children for medulloblastoma: correlation with IQ.

Treatment of children with cranial-spinal radiation (CSR) for brain tumors is associated with adverse intellectual outcome and white matter damage. However, the correlation between IQ and measures of white matter integrity has received little attention. We examined apparent diffusion coefficient (ADC), fractional anisotropy (FA), and intelligence in pediatric patients treated with CSR for medulloblastoma relative to control subjects. ADC and FA measures were obtained for eight patients and eight control children and evaluated in multiple regions of interest in the cerebral hemispheres. Mean ADC and mean FA for each region were calculated, group differences were evaluated, and the relationship between these measures and intelligence were examined. In our study group, decreased IQ was associated with increased ADC and decreased FA (P < 0.01). Mean IQ for the CSR group was lower than that for the control group, but the difference was not significant when controlling for overall mean FA or ADC (P > 0.10). Overall mean FA was lower and ADC was higher in the CSR group relative to controls (P < 0.01). Specifically, FA was lower in the genu of the corpus callosum, the anterior and posterior limbs of the internal capsule, inferior frontal white matter, and high frontal white matter, and ADC was higher in all regions in patients relative to controls (P < 0.01). Compromised white matter integrity was observed for multiple regions within the cerebral hemispheres following CSR. A novel finding was that microscopic damage in normal-appearing white matter, as indexed by higher ADC and lower FA, was related to poor intellectual outcome relative to age-matched controls.

Cerebellar Neoplasms↗
PubMed1986

Occipital lobe infarction caused by tentorial herniation.

Occipital lobe infarction caused by tentorial herniation was described based on computed tomography findings in nine patients. The whole area of the occipital lobe was involved in five patients; some areas were spared in the others. Infarction other than the ipsilateral occipital lobe was seen in four areas of nine patients: the ispsilateral posterior limb of the internal capsule, contralateral Ammon's horn, and two contralateral occipital lobes. Hemorrhagic infarction was seen in two patients.

Adolescent↗
PubMed1988

Magnetic resonance imaging stereotactic thalamotomy: report of a case with comparison to computed tomography.

The anatomical location of the thalamic target (ventrolateral nucleus) during stereotactic thalamotomy for a movement disorder was determined using magnetic resonance imaging (MRI) to define the anterior and posterior commissures and the intercommissural plane. Precise targeting was confirmed by intraoperative stereotactic computed tomography (CT), electrophysiological stimulation, and a gratifying postoperative response (disappearance of contralateral tremor and rigidity). The use of a MRI- and CT-compatible stereotactic coordinate frame allowed multiplanar imaging with excellent spatial and contrast resolution, visualization of the source of the tremor (prior embolic stroke affecting the dentatorubrothalamic pathway), and correction for the more medial location of the internal capsule in this patient. Location did not vary among target sites seen with MRI and CT imaging techniques. In this patient MRI during stereotactic thalamotomy supplemented CT and the electrophysiological technique that we conventionally use to define the ventrolateral nucleus. Although the importance of possible magnetic susceptibility imaging artifacts remains to be elucidated, stereotactic MRI may prove sufficiently accurate in the future to replace other imaging techniques used during functional neurosurgery.

Cerebrovascular Disorders↗
PubMed1989

Immunohistochemistry of human malignant astrocytoma cells xenografted to rat brain: apolipoprotein E.

Fresh xenografted human malignant astrocytoma cells migrate throughout the host rat brain. Cells from three Grade 3 human malignant astrocytomas were prelabeled with Phaseolus vulgaris leucoagglutinin (PHAL) and then xenografted into implantation pockets in rat host cerebral cortex. The human malignant astrocytoma cells in the host brain were immunocytochemically double-labeled for the presence of PHAL, which is used as a marker for graft derived cells, and either glial fibrillary acidic protein (GFAP), a specific marker for astrocytes and astrocytoma cells, or apolipoprotein E (APOE) 7 days, 14 days, 21 days, and 1 month later. Fresh human malignant astrocytoma cells (Grade 3 and 4) contained APOE and GFAP. The xenografted cells preserved APOE and GFAP in the host. PHAL double-labeled human malignant astrocytoma cells were found on the glia limitans along the entire circumference of the brain, in the corpus callosum, internal capsule, entopeduncular nucleus, optic tract, and median eminence. In addition, astrocytoma cells were observed in the cingulum, habenula, arcuate, and supraoptic nucleus. Astrocytoma cells entered the space of Virchow-Robin, migrated along parenchymal blood vessels and between the ependymal and subependymal layers of the third and lateral ventricles. APOE was a consistent marker for the migrating human malignant astrocytoma cells, but not an exclusive marker of the xenografted cells, since host rat reactive astrocytes also expressed APOE.

Animals↗
PubMed1994

Endoscopic resection of a subthalamic cavernous angioma: technical case report.

Endoscopic resection of a cavernous angioma at the subthalamic region was performed in a 33-year-old woman who presented with diplopia and progressive hemiparesis caused by recurrent hemorrhages. The entire surgical procedure was done through an 8-mm guiding tube inserted stereotactically from a frontal burr hole via an approach that traversed the anterior limb of the internal capsule. The patient's symptoms improved after surgery. This is believed to be the first report describing successful resection of a cavernous angioma located at the subthalamic region.

Adult↗
PubMed1995

Radiobiology of radiosurgery for refractory anxiety disorders.

The neuroradiological manifestations of bilateral single-session gamma (gamma)-irradiation to normal tissue contained in the internal capsule after gamma knife capsulotomy for otherwise intractable anxiety disorders were studied. In nine consecutive patients, a target maximum dose of 200 Gy was administered in a target volume of 276 +/- 42 mm3 (mean +/- SD) within the 50% isodose level. Serial computed tomographic and magnetic resonance imaging scans were undertaken from 3 to 44 months after irradiation. After surgery, a necrotic lesion appeared on computed tomographic scans, reaching its maximum volume (900 +/- 800 mm3) at 6 to 9 months, then decreasing (to 457 +/- 400 mm3) over the first postoperative year. This volume correlated with the mean isodose level of 91 (range, 41-143) Gy. On T2-weighted magnetic resonance imaging scans, the reaction tissue volumes were considerably larger and took longer to disappear than expected. In 15 targets, maximum reaction volumes were recorded at 1 to (approximately) 2 years after irradiation. In the remaining seven targets, smaller reaction volumes were observed, with no clear maxima appearing during 3 years of observation. In a pilot case, a lower target maximum dose of 160 Gy and a radiation volume of 275 mm3 within the 50% isodose gave only minimal surrounding tissue reactions. This report serves to alert clinicians that the tissue reaction volumes and the time course of their development after high irradiation doses may be less predictable than expected from previous observations in smaller radiation volumes. For this reason, lower irradiation doses and smaller volumes should be used in the future, and the time factor should be taken into account when interpreting computed tomographic and magnetic resonance images of gamma-knife-induced lesions.

Adult↗