Combining quantitative susceptibility mapping to the morphometric index in differentiating between progressive supranuclear palsy and Parkinson's disease.

PURPOSE: To determine whether the susceptibility value in the deep gray matter obtained by quantitative susceptibility mapping (QSM) provides additive value to the morphometric index for differentiating progressive supranuclear palsy (PSP) from Parkinson's disease (PD). MATERIALS AND METHODS: PSP- (n = 8) and PD patients (n = 18) and 18 age-matched healthy controls who underwent QSM and 3D magnetization-prepared rapid gradient echo (MPRAGE) sequences. The mean susceptibility values (MSVs) of the deep gray matter structures on QSM- and areas of the midbrain (morphometric index, MI) on 3D MPRAGE images were measured by two neuroradiologists. Analysis of variance, the Scheffe test and receiver operating characteristic (ROC) analysis were conducted to assess differences and discriminate among PSP, PD and controls by the MSVs and the MI. Using the MSV of a structure with the best area under the curve (AUC) and the MI, we created a decision tree to differentiate between PSP and PD. RESULTS: The MSVs of the globus pallidus (GP) and substantia nigra (SN) were significantly higher in PSP than PD and the controls (p < .05). By ROC analysis (PSP vs PD), AUC was greatest (0.903) for the GP. The MI was significantly smaller in PSP than PD and the controls (p < .05); AUC (PSP vs PD) was 0.917. The decision tree using cutoff values of 244 parts per billion for MSV of the GP and 74.0 mm2 for MI served to completely differentiate between PSP and PD. CONCLUSION: The MSV in the GP on QSM images adds value to the MI for differentiating PSP from PD.

Brain-wide Mapping of Mono-synaptic Afferents to Different Cell Types in the Laterodorsal Tegmentum.

The laterodorsal tegmentum (LDT) is a brain structure involved in distinct behaviors including arousal, reward, and innate fear. How environmental stimuli and top-down control from high-order sensory and limbic cortical areas converge and coordinate in this region to modulate diverse behavioral outputs remains unclear. Using a modified rabies virus, we applied monosynaptic retrograde tracing to the whole brain to examine the LDT cell type specific upstream nuclei. The LDT received very strong midbrain and hindbrain afferents and moderate cortical and hypothalamic innervation but weak connections to the thalamus. The main projection neurons from cortical areas were restricted to the limbic lobe, including the ventral orbital cortex (VO), prelimbic, and cingulate cortices. Although different cell populations received qualitatively similar inputs, primarily via afferents from the periaqueductal gray area, superior colliculus, and the LDT itself, parvalbumin-positive (PV+) GABAergic cells received preferential projections from local LDT neurons. With regard to the different subtypes of GABAergic cells, a considerable number of nuclei, including those of the ventral tegmental area, central amygdaloid nucleus, and VO, made significantly greater inputs to somatostatin-positive cells than to PV+ cells. Diverse inputs to the LDT on a system-wide level were revealed.

Parálisis nuclear del III par craneal en el contexto de un traumatismo craneoencefálico / Nuclear paralysis of the oculomotor nerve within the context of traumatic brain injury

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A probabilistic template of human mesopontine tegmental nuclei from in vivo 7T MRI.

Mesopontine tegmental nuclei such as the cuneiform, pedunculotegmental, oral pontine reticular, paramedian raphe and caudal linear raphe nuclei, are deep brain structures involved in arousal and motor function. Dysfunction of these nuclei is implicated in the pathogenesis of disorders of consciousness and sleep, as well as in neurodegenerative diseases. However, their localization in conventional neuroimages of living humans is difficult due to limited image sensitivity and contrast, and a stereotaxic probabilistic neuroimaging template of these nuclei in humans does not exist. We used semi-automatic segmentation of single-subject 1.1mm-isotropic 7T diffusion-fractional-anisotropy and T2-weighted images in healthy adults to generate an in vivo probabilistic neuroimaging structural template of these nuclei in standard stereotaxic (Montreal Neurological Institute, MNI) space. The template was validated through independent manual delineation, as well as leave-one-out validation and evaluation of nuclei volumes. This template can enable localization of five mesopontine tegmental nuclei in conventional images (e.g. 1.5T, 3T) in future studies of arousal and motor physiology (e.g. sleep, anesthesia, locomotion) and pathology (e.g. disorders of consciousness, sleep disorders, Parkinson's disease). The 7T magnetic resonance imaging procedure for single-subject delineation of these nuclei may also prove useful for future 7T studies of arousal and motor mechanisms.

Secondary paroxysmal dyskinesia in multiple sclerosis: Clinical-radiological features and treatment. Case report of seven patients.

Secondary paroxysmal dyskinesias (SPDs) are short, episodic, and recurrent movement disorders, classically related to multiple sclerosis (MS). Carbamazepine is effective, but with risk of adverse reactions. We identified 7 patients with SPD among 457 MS patients (1.53%). SPD occurred in face ( n = 1), leg ( n = 2), or arm +leg ( n = 4) several times during the day. Magnetic resonance imaging (MRI) showed new or enhancing lesions in thalamus ( n = 1), mesencephalic tegmentum ( n = 1), and cerebellar peduncles ( n = 5). Patients were treated with clonazepam and then acetazolamide ( n = 1), acetazolamide ( n = 5), or levetiracetam ( n = 1) with response within hours (acetazolamide) to days (levetiracetam). No recurrences or adverse events were reported after a median follow-up of 33 months.

Extended Anatomical Grading in Diffuse Axonal Injury Using MRI: Hemorrhagic Lesions in the Substantia Nigra and Mesencephalic Tegmentum Indicate Poor Long-Term Outcome.

Clinical outcome after traumatic diffuse axonal injury (DAI) is difficult to predict. In this study, three magnetic resonance imaging (MRI) sequences were used to quantify the anatomical distribution of lesions, to grade DAI according to the Adams grading system, and to evaluate the value of lesion localization in combination with clinical prognostic factors to improve outcome prediction. Thirty patients (mean 31.2 years ±14.3 standard deviation) with severe DAI (Glasgow Motor Score [GMS] <6) examined with MRI within 1 week post-injury were included. Diffusion-weighted (DW), T2*-weighted gradient echo and susceptibility-weighted (SWI) sequences were used. Extended Glasgow outcome score was assessed after 6 months. Number of DW lesions in the thalamus, basal ganglia, and internal capsule and number of SWI lesions in the mesencephalon correlated significantly with outcome in univariate analysis. Age, GMS at admission, GMS at discharge, and low proportion of good monitoring time with cerebral perfusion pressure <60 mm Hg correlated significantly with outcome in univariate analysis. Multivariate analysis revealed an independent relation with poor outcome for age (p = 0.005) and lesions in the mesencephalic region corresponding to substantia nigra and tegmentum on SWI (p = 0.008). We conclude that higher age and lesions in substantia nigra and mesencephalic tegmentum indicate poor long-term outcome in DAI. We propose an extended MRI classification system based on four stages (stage I-hemispheric lesions, stage II-corpus callosum lesions, stage III-brainstem lesions, and stage IV-substantia nigra or mesencephalic tegmentum lesions); all are subdivided by age (≥/<30 years).

Lateral inferior cerebellar peduncle approach to dorsolateral medullary cavernous malformation.

OBJECT: Brainstem cavernous malformations (BSCMs) present a unique therapeutic challenge to neurosurgeons. Resection of BSCMs is typically reserved for lesions that reach pial or ependymal surfaces. The current study investigates the lateral inferior cerebellar peduncle as a corridor to dorsolateral medullary BSCMs. METHODS: In this retrospective review, the authors present the cases of 4 patients (3 women and 1 man) who had a symptomatic dorsolateral cavernous malformation with radiographic and clinical evidence of hemorrhage. RESULTS: All patients underwent excision of the cavernous malformation via a far-lateral suboccipital craniotomy through the foramen of Luschka and with an incision in the inferior cerebellar peduncle. On intraoperative examination, 2 of the 4 patients had hemosiderin staining on the surface of the peduncle. All lesions were completely excised and all patients had a good or excellent outcome (modified Rankin Scale scores of 0 or 1). CONCLUSIONS: This case series illustrates that intrinsic lesions of the dorsolateral medulla can be safely removed laterally through the foramen of Luschka and the inferior cerebellar peduncle.

Interpeduncular arachnoid cysts in infants and children: insight into the entity based on a case series with long-term follow-up.

OBJECTIVE: Arachnoid cysts occupying the suprasellar region comprise 10-15% of intracranial distribution. Unlike large suprasellar cysts, pure interpeduncular cysts (IPCs) are rare, and their natural history is unknown. We describe a small series of children diagnosed with IPC and their long-term natural history. METHODS: A retrospective review was conducted of interpeduncular arachnoid cysts diagnosed over the years 2000-2010 at our center. Patients with clearly suprasellar cysts were excluded. Serial magnetic resonance imaging and long-term follow-up examinations were analyzed. Additionally, we conducted an extensive literature review focusing on the differences between suprasellar cysts and IPCs. RESULTS: We identified three pediatric patients with "pure" IPC; all of these had a follow-up of more than 5 years, and none was operated. Only six additional cases were identified in the literature. In both our experience and in the literature review, IPCs proved stable over the course of time, both radiologically as well as clinically. CONCLUSIONS: The clinical and radiological features of IPCs are not well defined. Variations in the relationship of arachnoid cysts in this area to Liliequist's membrane may explain the different subgroups that have been identified as well as the confusing nomenclature. IPCs are usually diagnosed as incidental findings or present with mild endocrine disorders. Associated findings of hydrocephalus, mass effect, and compression of neighboring structures, such as the chiasm, are not as frequent as with suprasellar cysts. Given the high likelihood of continuing stability, a conservative strategy of follow-up is recommended for pure IPCs that demonstrate preservation of the third ventricle.

Deep brain stimulation in cluster headache: hypothalamus or midbrain tegmentum?

Functional and structural neuroimaging studies have provided pivotal insights into the pathophysiology of trigeminal autonomic cephalalgias (TACs), particularly cluster headache (CH). Functional imaging studies using positron emission tomography (PET) and functional magnetic resonance imaging (fMRI) in TACs have reported activation of the posterior hypothalamus. A structural neuroimaging study using voxel-based morphometry in CH reported increased volume of the hypothalamic gray, although another larger study failed to reproduce this finding. These studies in CH prompted the use of stereotactic stimulation of the target point identified by functional and structural neuroimaging. The precise anatomical localization of the deep brain stimulation (DBS) target places it at the midbrain tegmentum rather than the posterior hypothalamus. A comparison of the PET and fMRI studies in TACs reveals that the diencephalic/mesencephalic activation is more posteroinferior in the PET studies, straddling the hypothalamus and midbrain tegmentum, whereas the activation is centered on the hypothalamus in the higher spatial resolution fMRI studies. To optimize the outcomes from DBS, it is likely that patients will need to be studied individually using functional imaging techniques that have high spatial and temporal resolution to enable targeting of the appropriate locus with stereotactic stimulation.

Thrombosis and subsequent recanalization of a ruptured intracranial aneurysm in 2 children, demonstrating the value of repeating catheter angiography after an initial negative study.

The cases of 2 children with true aneurysmal subarachnoid hemorrhages (SAHs) and initial false-negative angiograms are reported. In both cases, the initial angiogram was of adequate technical quality and included the projections on which aneurysms were later documented. There was no significant vasospasm at the time of initial angiography; therefore, transient aneurysm sac thrombosis was the most likely explanation for the initial false-negative studies. It is particularly interesting to note that 1 of the 2 patients had a pattern of hemorrhage compatible with the most limited definition of a perimesencephalic SAH, that is, a small prepontine cistern hemorrhage. If a second angiogram had been deemed unnecessary based on that criterion alone, a ruptured basilar tip aneurysm would have escaped detection and treatment.

Post-traumatic interpeduncular cistern hemorrhage as a marker for brainstem lesions.

We retrospectively reviewed a prospectively collected database of our diffuse axonal injury (DAI) patients to evaluate the accuracy of the evidence of interpeduncular cistern (IPC) blood on computed tomography (CT) scan when diagnosing brainstem lesions (BSL) early after trauma. From December 1989 to December 2008 we prospectively maintained a clinical and radiological database of head injured patients admitted to our neurosurgical intensive care unit (ICU) that met the following criteria: coma (Glasgow Coma Scale [GCS] score < 9) following the traumatic event; neurological derangement not ascribable to hypoxia, hypotension, or long-acting drugs able to alter state of consciousness; absence of lesions accounting for the severity of coma either on the admission CT scan or on subsequent CT scans; and no contraindications to magnetic resonance imaging (MRI; e.g., indwelling metallic implants). Patients with MRI evidence of BSL exhibited a significantly higher incidence of IPC blood on CT scan than patients without such evidence (77.92% versus 20.00%; p < 0.0001). However, these same patients showed a similar incidence of lesions not associated with IPC blood (68.83% versus 56%; p = 0.2459). The evidence of IPC blood on CT scan as an indicator of BSL had a sensitivity of 0.78 (95% CI: 0.70, 0.86), and a specificity of 0.80 (95% CI: 0.72, 0.88), with a 3.90 likelihood ratio for a positive CT scan, and a 0.28 likelihood ratio for a negative CT scan. Our data suggest that the finding of IPC blood on CT scan early after trauma in patients with otherwise unexplained coma is a good marker for possible brainstem lesions.

[Epidural abscess and encephalocele following tegmen defect due to mastoidectomy: a case report]. / Mastoidektomiye bagli tegmen defekti sonrasi gelisen epidural apse ve ensefalosel: Olgu sunumu.

In chronic otitis media surgery, especially in cases with cholesteatoma, different complications can occur in the course of the operation or postoperatively. In our clinic, in one of the cases who had staged canal-wall down operation for otitis media with cholesteatoma, an iatrogenic 0.5x0.5 cm dural plate defect had occurred in tegmen tympani during the operation. In this article, we present an uncommon case who had encephalocele with epidural abscess because of unrepaired dural plate defect superimposed with early postoperative infection at the first month after the operation and we discuss it in the light of the literature.

Dystonia associated with pontomesencephalic lesions.

Secondary dystonia is well known subsequent to lesions of the basal ganglia or the thalamus. There is evidence that brainstem lesions may also be associated with dystonia, but little is known about pathoanatomical correlations. Here, we report on a series of four patients with acquired dystonia following brainstem lesions. There were no basal ganglia or thalamic lesions. Three patients suffered tegmental pontomesencephalic hemorrhage and one patient diffuse axonal injury secondary to severe craniocerebral trauma. Dystonia developed with a delay of 1 to 14 months, at a mean delay of 6 months. The patients' mean age at onset was 33 years (range 4-56 years). All patients presented with hemidystonia combined with cervical dystonia, and two patients had craniofacial dystonia in addition. Three patients had postural or kinetic tremors. Dystonia was persistent in three patients, and improved gradually in one. There was little response to medical treatment. One patient with hemidystonia combined with cervical dystonia improved after thalamotomy. Overall, the phenomenology of secondary dystonia due to pontomesencephalic lesions is similar to that caused by basal ganglia or thalamic lesions. Structures involved include the pontomesencephalic tegmentum and the superior cerebellar peduncles. Such lesions are often associated with fatal outcome. While delayed occurrence of severe brainstem dystonia appears to be rare, it is possible that mild manifestations of dystonia might be ignored or not be emphasized in the presence of other disabling deficits.

Frontal-lobe syndrome and psychosis after damage to the brainstem dopaminergic nuclei.

A patient developed frontal-lobe syndrome and psychotic symptoms after infarction in the pontomesencephalic junction. Stereotaxic lesion localization on magnetic resonance imaging and statistical analyses of regional cerebral blood flow (rCBF) disclosed an involvement of the rostral brainstem dopaminergic nuclei and hypoperfusion in the frontal-subcortical circuit components. We suggest that the patient's cognitive and behavioral disturbances were associated with disruption of ascending dopaminergic projections to the frontal-subcortical circuits.

The metabolic topography of posthypoxic myoclonus.

Posthypoxic myoclonus (PHM) is a syndrome of action and intention myoclonus that occurs in some patients who survive a cardiac arrest. Using PET and statistical parametric mapping, the authors observed a significant bilateral increase in glucose metabolism in the ventrolateral thalamus and pontine tegmentum in patients relative to controls. Interventions such as deep brain stimulation that interrupt networks that involve these structures may be useful in patients with severe PHM.

Regional cerebral blood flow in fibromyalgia: single-photon-emission computed tomography evidence of reduction in the pontine tegmentum and thalami.

OBJECTIVE: To determine whether regional cerebral blood flow (rCBF) is abnormal in any cerebral structure of women with fibromyalgia (FM), following a report that rCBF is reduced in the thalami and heads of caudate nuclei in FM. METHODS: Seventeen women with FM and 22 healthy women had a resting single-photon-emission computed tomography (SPECT) brain scan to assess rCBF and a T1-weighted magnetic resonance imaging (MRI) scan to enable precise anatomic localization. Additionally, all participants underwent 2 manual tender point examinations and completed a set of questionnaires evaluating clinical features. SPECT scans were analyzed for differences in rCBF between groups using statistical parametric mapping (SPM) and regions of interest (ROIs) manually drawn on coregistered MRI. RESULTS: Compared with control subjects, the rCBF in FM patients was significantly reduced in the right thalamus (P = 0.006), but not in the left thalamus or head of either caudate nucleus. SPM analysis indicated a statistically significant reduction in rCBF in the inferior pontine tegmentum (corrected P = 0.006 at the cluster level and corrected P = 0.023 for voxel of maximal significance), with consistent findings from ROI analysis (P = 0.003). SPM also detected a reduction in rCBF on the perimeter of the right lentiform nucleus. No correlations were found with clinical features or indices of pain threshold. CONCLUSION: Our finding of a reduction in thalamic rCBF is consistent with findings of functional brain imaging studies of other chronic clinical pain syndromes, while our finding of reduced pontine tegmental rCBF is new. The pathophysiologic significance of these changes in FM remains to be elucidated.

Comparison of impaired subcortico-frontal metabolic networks in normal aging, subcortico-frontal dementia, and cortical frontal dementia.

Normal aging, progressive supranuclear palsy (PSP), and frontotemporal dementia (FTD) are characterized by different degrees of decline in frontal lobe functions. We used (18)FDG-PET and statistical parametric mapping (SPM96) to compare relative subcorticofrontal metabolic impairment at rest in 21 healthy elderly subjects (HES), 20 PSP patients, and 6 FTD patients. When HES were compared to 22 healthy young subjects, widespread decrease in metabolism was observed in bilateral medial prefrontal areas including anterior cingulate cortices, in dorsolateral prefrontal areas, in left lateral premotor area, in Broca's area, and in left insula. In PSP compared to the 43 healthy subjects (HS), we observed subcorticofrontal metabolic impairment including both motor and cognitive neural networks. Impairment of functional connections between midbrain tegmentum and cerebellar, temporal and pallidal regions was demonstrated in PSP as compared to HS. When comparing FTD to HS, glucose uptake was primarily reduced in dorsolateral and ventrolateral prefrontal cortices and in frontopolar and anterior cingulate regions. There was also bilateral anterior temporal, right inferior parietal, and bilateral striatal hypometabolism. Finally, FTD showed more severe striatofrontal metabolic impairment than PSP, while mesencephalothalamic involvement was only observed in PSP. Our data suggest that subcorticofrontal metabolic impairment is distributed in distinct subcorticocortical networks in normal aging, PSP, and FTD. Subcorticofrontal dementia in PSP is related to hypometabolism in discrete frontal areas, which are probably disconnected from certain subcortical structures. The concept of subcortical dementia is reinforced by our data, which show disrupted functional connections between mesencephalon and cerebellar cortex, inferior and medial temporal regions, and pallidum.

MR vs CT in progressive supranuclear palsy.

Nine progressive supranuclear palsy (PSP) patients were studied with computerized tomography (CT) and magnetic resonance (MR) in order to determine the efficacy of each in detecting atrophy of the brainstem. Three additional PSP patients were evaluated with MRI for quantitative (electronic) measurements of the colliculi, pons and midbrain tegmentum. Both CT and MRI were equally effective in demonstrating midbrain atrophy. The MR was able to utilize the sagittal view to visualize thinning of the collicular (quadrigeminal) plate, a useful sign in PSP. Atrophy of the thinned collicular plate is more pronounced in the superior colliculus, one of the most common sites of pathology in PSP. The MR is able to make quantitative measurements of the degree of atrophy of the colliculi, pons and midbrain tegmentum.