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PubMed2006

Magnetization transfer ratio and volumetric analysis of the brain in macrocephalic patients with neurofibromatosis type 1.

The purpose of the study was to evaluate brain myelination by measuring the magnetization transfer ratio (MTR) and to measure grey (GMV) and white matter volume (WMV) in macrocephalic children with neurofibromatosis type 1 (NF1). Seven NF1 patients (aged 0.65-16.67 years) and seven age- and gender-matched controls were studied. A three-dimensional (3D) gradient echo sequence with and without magnetization transfer (MT) prepulse was used for MTR assessment. Volume measurements of GM and WM were performed by applying segmentation techniques on T2-weighted turbo spin echo images (T2WI). MTR of unidentified bright objects (UBOs) on T2WI in cerebellar white matter (52.8+/-3.3), cerebral peduncles (48.5+/-1.5), hippocampus (52.6+/-1.1), internal capsule (55.7+/-0.3), globus pallidus (52.7+/-3.9), and periventricular white matter (52.6+/-1.2) was lower than in the corresponding areas of controls (64.6+/-2.5, 60.8+/-1.3, 56.4+/-0.9, 64.7+/-1.9, 59.2+/-2.3, 63.6+/-1.7, respectively; p<0.05). MTR of normal-appearing brain tissue in patients was not significantly different than in controls. Surface area (mm(2)) of the corpus callosum (809.1+/-62.8), GMV (cm(3)) (850.7+/-42.9), and white matter volume (WMV) (cm(3)) (785.1+/-85.2) were greater in patients than in controls (652.5+/-52.6 mm(2), 611.2+/-92.1 cm(3), 622.5+/-108.7 cm(3), respectively; p<0.05). To conclude, macrocephaly in NF1 patients is related to increased GMV and WMV and corpus callosum enlargement. MTR of UBOs is lower than that of normal brain tissue.

Adolescent↗
PubMed1998

Spiral CT vs incremental CT: is spiral CT superior in imaging of the brain?

The aim of this study was to evaluate image quality of spiral CT of the brain as compared with incremental CT using identical scanning parameters. Incremental or spiral cranial CT was performed on 46 consecutive, randomized patients with non-traumatic disease of the brain on a Siemens (Erlangen, Germany) Somatom Plus 4. Evaluation was done in a randomized blinded way by two experienced radiologists. Different anatomical structures, image noise, and artifacts were scaled 1 (bad) to 4 (very good). Statistical analysis was done using the F-test of variance for partial sums of squares as well as Student's t-test. Incremental CT was superior to spiral CT for evaluation of the internal capsule, supratentorial artifacts, gray/white matter differentiation, and image noise. No statistically significant differences were seen for evaluation of the pons, infratentorial artifacts, and eye muscles. With identical scanning parameters incremental CT is superior to spiral CT in the assessment of small, complex structures in a low-contrast setting. No differences are seen for larger structures or small structures in a medium-contrast range. Artifacts localized close to the skull in spiral CT can easily mimic hemorrhage in traumatized patients. Spiral CT should therefore only be used for CT angiography and if 3D reconstructions are needed.

Artifacts↗
PubMed2006

Acute necrotizing encephalopathy: combined therapy and favorable outcome in a new case.

BACKGROUND: Acute necrotizing encephalopathy (ANE) is a rare disease characterized by multiple, symmetrical brain lesions, affecting thalami, brainstem tegmentum, and cerebellar medulla; more inconstantly, other structures are involved, i.e., internal capsules, posterolateral putamen, and deep periventricular white matter. FEATURES: The clinical picture consists of rapidly deteriorating acute monophasic encephalopathy preceded by prodromal febrile illness; the symptoms include hyperpyrexia, convulsions, recurrent vomiting, and coma within 24 h. PROGNOSIS: The outcome is usually poor and approximately 70% of the patients die within a few days from the onset of fever. There is no specific therapy for ANE but, in some patients, the clinical status improved with steroid treatment.

Child, Preschool↗
PubMed2001

Neuropathology of Raine syndrome.

We present three cases of Raine syndrome occurring in siblings of consanguineous parents. Raine syndrome is characterised by generalised osteosclerosis with craniofacial anomalies and intracranial calcifications. So far, only nine cases have been reported, and no evaluation of the distribution and extent of the cerebral mineralisations, as well as their impact on the surrounding tissue, has been undertaken yet. In our cases, calcifications were unevenly distributed throughout the central nervous system, not associated with neuronal loss or dystrophic events and appeared mostly as single calcospherites within the neuropil with occasional confluent deposits at advanced gestational age. There was intense perifocal microgliosis around single immature calcospherites, as well as mild astrogliosis around and within the confluent lesions, in which occasional macrophages could be found. Rarely, mineralisations occurred in blood-vessel walls, mainly affecting basal ganglia. Preferential sites of calcification were parietal and occipital periventricular white matter and corpus callosum, while frontal lobes were mildly affected. The cortex, temporal lobes as well as internal capsule, brain stem, cerebellum, leptomeninges, pituitary gland and choroid plexus were devoid of mineralisations. The subcortical grey matter was moderately involved in the putamen and pallidum, mildly in the caudate nucleus and subependymal germ cell matrix and not at all in the thalamus, Ammon's horn, amygdala and substantia nigra. The distribution of mineral deposits was thus inversely correlated to regional blood circulation and capillary density, with calcifications being concentrated in more sparsely perfused areas but lacking in highly vascularised tissue. This inverse relationship between mineralisation and regional blood flow was reflected in the varying distribution of calcospherites in grey and white matter as well as in the white matter of different lobes.

Brain Diseases↗
PubMed2003

Anomalous alterations affecting microglia in the central nervous system of a fetus at 12 weeks of gestation: case report.

We report here on the first documented case of profound alterations specifically affecting the microglial population within the nervous system during the fetal period. This case, derived at gestational week 12, was one amongst a series of second trimester brains currently being investigated with respect to microglial colonization of the human fetal brain. No significant pathological alterations could be identified upon gross macroscopy or following microscopic analysis of serial brain sections stained with cresyl fast violet (Nissl). By contrast, sections stained immunohistochemically to detect MHC class II (CR3/43) and CD68 (PG-M1) antigens revealed a marked pathological change in the morphology and density of microglia within the CNS. Specifically, labeled cells within the rostral telencephalon were clearly hypertrophied and emitted numerous, branched processes in all directions, appearing in an atypical 'hyper-ramified' state uncharacteristic of microglia found in normal brains at this age. However, cells located elsewhere in the CNS (for example in the thalamus and internal capsule) appeared in a less differentiated state (small, rounded cells lacking processes) when compared to those within normal age-matched control brains. The total density and distribution of these labeled cells far outnumbered that seen in normal development. As far as we are aware, such an anomaly specifically affecting microglia, has not been documented previously. Consequently, this case represents the first of its kind, and the remarkable observations outlined in this study bear considerable significance from a neuropathological standpoint for future investigations into pathological changes affecting microglia in the central nervous system during the fetal period.

Antigens, CD↗
PubMed1999

Increased density of oligodendrocytes in childhood ataxia with diffuse central hypomyelination (CACH) syndrome: neuropathological and biochemical study of two cases.

We report neuropathological, biochemical and molecular studies on two patients with childhood ataxia with diffuse central nervous system hypomyelination (CACH) syndrome, a leukodystrophy recently defined according to clinical and radiological criteria. Both had severe cavitating orthochromatic leukodystrophy without atrophy, predominating in hemispheric white matter, whereas U-fibers, internal capsule, corpus callosum, anterior commissure and cerebellar white matter were relatively spared. The severity of white matter lesions contrasted with the rarity of myelin breakdown products and astroglial and microglial reactions. In the white matter, there was an increase in a homogeneous cell population with the morphological features of oligodendrocytes, in many instances presenting an abundant cytoplasm like myelination glia. These cells were negative for glial fibrillary acidic protein and antibodies PGM1 and MIB1. Some were positive for myelin basic protein, proteolipid protein (PLP), and myelin oligodendrocyte glycoprotein, but the majority were positive for human 2'-3' cyclic nucleotide 3' phosphodiesterase and all were positive for carbonic anhydrase II, confirming that they are oligodendrocytes. Myelin protein and lipid content were reduced. The PLP gene, analyzed in one case, was not mutated or duplicated. The increased number of oligodendrocytes without mitotic activity suggests an intrinsic oligodendroglial defect or an abnormal interaction with axons or other glial cells. This neuropathological study supports the notion that CACH syndrome constitutes a specific entity.

Ataxia↗
PubMed2004

Combined MR spectroscopic imaging and diffusion tensor MRI visualizes corticospinal tract degeneration in amyotrophic lateral sclerosis.

Motor neuron damage and cortical spinal tract (CST) degeneration are pathological features of amyotrophic lateral sclerosis (ALS). We combined whole-brain diffusion tensor imaging (DTI) and three-dimensional magnetic resonance spectroscopic imaging (MRSI) to study the CST at different locations. Eight ALS patients were compared with normal controls. Fractional anisotropy (FA) and mean diffusivity (MD), and the ratio of N-acetyl-aspartate (NAA) to creatine (Cr) were measured at various locations in the CST, including the subcortical white matter (SWM), centrum semiovale (CS), periventricular white matter (PV), posterior limb of the internal capsule (PIC) and cerebral peduncle (CP). Patients showed significantly lower FA than controls in the CST, including the SWM, CS, PV and PIC. Although there was a trend towards elevated MD in ALS patients, this did not reach statistical significance. NAA/Cr ratios were also decreased in ALS patients compared with normal controls, with significant differences in the SWM and PV but not in PIC. Combined whole-brain DTI and MRSI can detect axonal degeneration in ALS. Measurements of FA obtained in the SWM, CS, PV and PIC, and NAA/Cr ratios in the SWM and PV yield the most robust results.

Adult↗
PubMed1997

Subcortical type cognitive impairment in herpes zoster encephalitis.

Nine immunocompetent patients with acute herpes zoster encephalitis (HZE) were studied with the help of neurological investigations. All patients were treated with acyclovir. Neuropsychological performance was compared with that of a group of 16 healthy controls. Computed tomography of the head showed infarct-like hypodense lesions in two patients, involving the internal capsule in one case and the temporoparietal cortex and white matter in another. Hypoperfusion shown by single photon emission computed tomography, mostly involving the frontal areas bilaterally, was seen in six of the seven patients examined. Hyperperfusion as seen in herpes simplex encephalitis was not encountered. One patient remained mildly demented, but all the other patients recovered relatively well. Neuropsychological examination after acyclovir treatment showed a decline in memory and speed of cognitive processes, without circumscribed neuropsychological deficits. Six of the nine patients showed behavioural disinhibition, and mood changes were also observed. Memory impairment in HZE was not as global or as severe as is described after encephalitis due to herpes simplex virus. In HZE both the brain perfusion pattern and the neuropsychological test profile showed features compatible with subcortical dysfunction.

Adult↗
PubMed1997

Differences in factors associated with silent and symptomatic MRI T2 hyperintensity lesions.

The factors and symptomatology associated with different types of hyperintensity lesions on MRI were investigated. The study population consisted of 139 subjects who were recruited from 450 outpatients who had a neurological diagnosis in 1994. The subjects underwent brain magnetic resonance imaging between 1994 and 1995 and were divided into three groups (control, asymptomatic, and symptomatic) on the basis of T2 hyperintensity lesions, as well as a history of or neurological signs of stroke, or both. The demographic characteristics and risk factors were studied, and the T2 hyperintensity lesions were analysed semi-quantitatively. Results showed that: (1) the control and asymptomatic groups did not differ in terms of risk factors and demographic characteristics with the exception of age; (2) the symptomatic group was characterized by a significantly higher incidence of hypertension and electrocardiographic abnormalities, as well as significantly more numerous risk factors when compared with the other two groups; (3) the symptomatic patients also had higher proportion of men and higher levels of systolic blood pressure and blood glucose than the control patients, and more frequent hypertriglyceridaemia and higher triglyceride level than the asymptomatic patients; (4) the symptomatic group had a greater lesion distribution in the posterior basal ganglia-internal capsule and the infratentorial regions than did the asymptomatic group. We concluded that the asymptomatic and symptomatic groups should not be considered identical entities.

Aged↗
PubMed1999

A follow-up study of cognitive impairment due to inferior capsular genu infarction.

Abulia, memory loss, other cognitive deficits, and behavioral changes consistent with dementia can follow an inferior capsular genu infarction, but only little is known about the time course of these disturbances. The present study describes the long-term outcome of cognitive defects in four patients with inferior capsular genu infarction who underwent a neuropsychological examination within 3 and 12 months of onset. Three patients had infarcts in the inferior genu of the left internal capsule and had similar symptoms in the acute phase: disorientation, memory loss, language impairment, and behavioral changes. The patient with right-side infarct showed memory impairment and behavioral changes. Three patients had deficits in one or more cognitive domains on the first assessment, but none was demented. By the second evaluation all subjects had improved. In two patients there were a moderate memory defect persisted and a language disturbance. Improvement in these disturbances during long-time follow-up demonstrates that there are alternative pathways that reestablish the functional connections damaged by the strategically located capsular genu infarct. Inferior capsular genu infarction is not a cause of persisting "strategic infarct dementia."

Aged↗
PubMed2000

MRI and motor evoked potential findings in nondisabled multiple sclerosis patients with and without symptoms of fatigue.

Fatigue is a common symptom of multiple sclerosis (MS) even in the early phases of the disease, when neurological disability is usually still not present. To investigate the pathophysiology of fatigue we compared neurophysiological (motor evoked potentials of the four limbs, MEPs) and brain magnetic resonance imaging (MRI) findings in two groups of nondisabled MS patients, those with (n=15) and those without (n=15) fatigue. Fatigue was assessed by an interview and scored by the Fatigue Severity Scale. The two groups were matched for sex, age, disease duration, Expanded Disability Status Scale score, pyramidal Functional System (FS) score, and depression score. MEPs were abnormal in five patients with fatigue and in one patient without fatigue. A significant association was found between the patient scores on the Fatigue Severity Scale, and the burden of MRI lesions (r=0.5; P< 0.005). Significantly higher parietal lobe (P< 0.05), internal capsule (P< 0.05), and periventricular trigone (P< 0.05) lesion loads were found in patients with fatigue than in those without. Our results agree with a central nervous system origin of fatigue in MS patients. This symptom might be a consequence either of a functional deafferentation of the cortex due to cortico-subcortical interconnection damage or of a demyelination in critical sites of the CNS, such as the cortico-spinal tract.

Adolescent↗
PubMed2004

The anatomy of the porcine subthalamic nucleus evaluated with immunohistochemistry and design-based stereology.

This study provides a light-microscopic description of the organization, morphology and number of neurons in the subthalamic nucleus (STN) of the Göttingen minipig. It is based on histological material stained with Nissl, Golgi and autometallographic techniques, and employs design-based stereological estimation of the total neuron number. The organization of several neurotransmitters in the STN has been evaluated in histological preparations stained for acetylcholinesterase (AChE) and immunostained for choline acetyltransferase (ChAT), tyrosine hydroxylase (TH), glutamic acid decarboxylase (GAD) and glutamate. In all of the stained preparations the STN appeared as a distinct lens-shaped structure located in the caudal diencephalon, medial to the internal capsule and ventrolateral to the zona incerta. Rostrally, the STN approached the globus pallidus pars interna, whereas caudally the ventromedial part of the STN was adjacent to the rostral part of the substantia nigra pars compacta (SNc), where some of the neurons of the two nuclei merged. The neurons in the STN had medium-sized (25-40 microm) ovoid or fusiform cell bodies, from which three to six large dendrites emanated in a direction predominantly parallel to the long axis of the STN. Immunohistochemistry revealed that most of the subthalamic neurons were glutamatergic and differed significantly in appearance from the large stellate TH-positive cells of the adjacent SNc. Numerous TH-positive bouton-rich fibers traversed the STN. The GAD-staining revealed a large number of terminals within the boundaries of the STN. The STN was highly AChE-positive, reflecting a prominent innervation by ChAT-positive terminals. The total number of subthalamic neurons in one hemisphere was estimated to be approximately 56,000. We conclude that the neuroarchitecture of the porcine STN is similar to primates, including humans, and appears well-suited for further studies examining the role of the STN in movement disorders.

Animals↗
PubMed2000

Thalamic astrocytomas: surgical anatomy and results of a pilot series using maximum microsurgical removal.

Deep-seated astrocytomas within the basal ganglia and the thalamus are considered unfavourable for microsurgical removal since the circumferential neighbourhood of critical structures limits radical resection. On closer assessment, the thalamus has a unique configuration within the basal ganglia. Its tetrahedric shape has 3 free surfaces and only the ventrolateral border is in contact with vital and critical functional structures, e.g. the subthalamic nuclei and the internal capsule. The purpose of the present study was to investigate the feasibility of maximum microsurgical removal in a series of intrinsic thalamic astrocytomas. 14 patients with intrathalamic astrocytomas grades I to 4 as diagnosed by previous stereotactic biopsy or intra-operative frozen section were selected for maximum microsurgical removal. The infratentorial supracerebellar approach from the contralateral side was used for 4 limited neoplasms of the pulvinar. For the other 10 larger and more extensive processes a parieto-occipital transventricular approach was chosen. Final histology gave the result of astrocytoma grade 1 or 2 in 4 patients, and of astrocytoma grade 3 or 4 in 10 patients. Postoperative MRI confirmed reduction of the tumor mass by 80 to 100% in 11 of 14 cases. Regional ancillary radiotherapy with 60 Gy was administered postoperatively for astrocytomas grades 3 and 4. Two patients operated on via the posterior transventricular approach had new postoperative partial hemianopia. Five of the 14 patients finally needed a ventriculo-peritoneal shunt. During the follow-up time of 6 to 52 months, tumor progression/recurrence was observed in 6 of the 10 high grade and none of the low grade neoplasms. The present pilot series demonstrates the feasibility of the microsurgical concept. Comparison with other treatment modalities, such as brachytherapy, requires future consideration.

Adolescent↗
PubMed2004

Parkinsonism following striatal infarcts: incidence in a prospective stroke unit cohort.

A number of case reports have highlighted the occurrence of parkinsonism following strategic infarcts affecting the basal ganglia but the prevalence of parkinsonism after striatal infarcts (SI) has not been assessed. Therefore, we evaluated the clinical features and prevalence of parkinsonism in a large series of patients admitted to the Stroke-Unit of the Department of Neurology Innsbruck. Cerebral scans were retrospectively screened for SI, defined as a lesion larger than 1.5 cm involving the basal ganglia and the internal capsule. Out of 622 patients, 27 met the criteria for SI (4.3%) and 11 patients were available for follow-up. All patients presented contralateral motor weakness. Bilateral akinetic-rigid parkinsonism was found in only one patient whose [(123)I]beta-CIT-SPECT showed a decrease of the ligand uptake following the limits of the vascular lesion. Overall, parkinsonism does not appear to be a frequent consequence of striatal infarcts. Multiple lacunar subcortical infarcts interrupting thalamocortical drive may be more critical for the development of vascular parkinsonism.

Aged↗
PubMed2006

Minor stroke and major vascular occlusion. A case report.

Occlusion of middle cerebral artery (MCA) is generally associated to severe stroke and poor prognosis; however a few patients with mild to moderate presentation and long-term reversibility of neurological deficits have been reported. A 66-year-old male presented with left-side weakness and dysarthria (NIHSS score 7), which progressively resolved within a few days; ischaemic lesion of the anterior arm of the right internal capsule was found at brain CT obtained 72 h after presentation. Transcranial Colour Doppler showed absence of flow of the right MCA. Cerebral angiography showed occlusion of the right MCA that was retrogradely revascularised by leptomeningeal collaterals. Non-invasive intracranial vascular examinations could identify major intracranial artery lesions in patients who present with mild to moderate stroke symptoms. These patients could be identified and followed to clarify their best treatment and prognosis.

Aged↗
PubMed1975

Efferent connections of the orbitofrontal cortex in the marmoset (Saguinus oedipus).

Unilateral partial ablations were made in the orbitofrontal cortex of 4 adult marmosets (Saguinus oedipus) and fiber degeneration was traced using the Nauta-Gygax and Fink-Heimer selective silver impregnation techniques. Corticocortical projections were found to the ipsilateral convexity and medial aspect of the frontal lobe and to the homologous orbitofrontal areas of the contralateral hemisphere. Fiber degeneration was followed through the uncinate fascicle to the temporal and insular cortices, and caudally into the rostrolateral entorhinal cortex. Other fibers joined the cingulum bundle and terminated throughout the cingulate cortex. Subcortical projections were observed to the lateral and basal amygdaloid nuclei, caudate head, ventrolateral putamen and ventral claustrum. The lateral preoptic and hypothalamic areas received a small number of fibers, as did the intralaminar and reticular thalamic nuclei. The dorsomedial nucleus of the thalamus was recipient of a large group of fibers which followed the ventral internal capsule and joined the inferior thalamic peduncle to terminate there. Preterminal debris appeared heaviest in the dorsomedial thalamic nucleus, pars magnocellularis (MDmc) in more caudal orbital lesions. A subthalamic projection to field H of Forel was observed. A small number of fibers terminated in the lateral midbrain tegmentum, but no appreciable fiber degeneration was observed more caudally than the midbrain. These results are compared in some areas to findings in the rhesus monkey. The possibility of a topical organization in the orbital cortical and thalamic projections is discussed.

Animals↗
PubMed1976

The efferent projections of the medial prefrontal cortex in the squirrel monkey (Saimiri sciureus).

Unilateral partial ablations were made in the medial granular frontal cortex of 6 adult squirrel monkeys. Fiber degeneration was traced using the Nauta-Gygax and Fink-Heimer selective silver impregnation techniques into the cingulum, uncinate fascicle, corpus callosum, internal capsule and sublenticular (ventral extracapsular) bundle. Corticocortical projections were observed to the lateral orbital, parietal, rostral inferior temporal, and entorhinal cortices, as well as to the hippocampus proper. Subcortical projections were observed to the basal amygdaloid nucleus, lateral hypothalamus, lateral dorsal, anterior ventral, mediodorsal, pulvinar, and intralaminar nuclei of the thalamus. Preterminal and terminal fiber degeneration were notably sparse in most of the neostriatum, except the dorsolateral region of the caudate nucleus. Fiber degeneration traversed the medial crus cerebri of the midbrain, where fibers of passage ascended into the tegmentum through the medial substantia nigra. Preterminal and terminal degeneration were present in the mesencephalic reticular formation lateral and dorsal to the red nucleus and a few degenerating fibers were followed into the dorsolateral periaqueductal gray. Degeneration was observed as far caudal as the reticulotegmental nucleus of the rostral pons. On the basis of its connections, the medial prefrontal cortex resembles the remainder of the prefrontal lobe and probably also has an important role in cortical and subcortical limbic mechanisms.

Amygdala↗
PubMed1976

The rodent neostriatum: a Golgi analysis.

In the adult rodent, coronal sections of Golgi impregnations of the neostriatum display a compact segregation of axon fascicles, neuronal clusters, and dendritic bundles thus forming an areolar configuration. Isolated neurons are rarely seen. The dorsomedial region of the neostriatum appears free of axon fascicles and dendritic bundles. Horizontal and sagittal sections of the neostriatum show clusters of cells parallel to axon fascicles. The neurons exhibit spine-laden dendrites with an initial spine-free segment. Neonatal impregnations exhibit a different configuration. Neonatally, cells tend to cluster but there is no bundling of dendrites. Neurons are spine-free or have protospines on the soma and the dendrites, including the initial segment. Transition from neonate to adult configuration is discernible at about 15 days after birth. The neostriatum of carnivores exhibits a different structure from the rodent neostriatum. This difference is associated with a developed anterior limb of the internal capsule in the carnivore. The axon fascicle-free portion of the carnivore neostriatum lacks dendritic bundles and pallisades. Portions near the capsule with axon fascicles appear similar to the rodent neostriatum with dendritic bundlings and pallisading. Such findings emphasize the importance of total neuronal configuration (neuronal-architectonics) in morphologic analyses.

Animals↗