A quantitative MRI index for assessing the severity of hippocampal sclerosis in temporal lobe epilepsy.

BACKGROUND: Hippocampal sclerosis (HS) is associated with post-surgery outcome in patients with temporal lobe epilepsy (TLE), and an automated method that quantifies HS severity is still lacking. Here, we aim to propose an MRI-based HS index (HSI) that integrates hippocampal volume and FLAIR signal to measure the severity of HS. METHODS: Forty-two pre-surgery TLE patients were included retrospectively, with T1-weighted (T1W) and FLAIR images acquired from each subject. Two experienced neurosurgeons (W.D. and C.S.) and one neurologist (Q.L.) rated HS severity with a four-class grading scale (normal, mild, moderate and severe) based on both hippocampal volume loss and increased FLAIR signal. A consensus of HS severity for each subject was made by voting among the three visual rating results. Regarding the automatic quantification, the hippocampal volume was quantified by AccuBrain on T1W image, and the FLAIR signal of hippocampus was calculated as the mean intensity of hippocampal region on the FLAIR image (normalized by the mean intensity of gray matter). To fit the HSI from visual rating, we applied ordinal regression with the voted visual rating as the dependent variable, and hippocampal volume and FLAIR signal as the independent variables. The HSI was calculated by weighting the predicted probabilities of the four-class grading scales from ordinal regression. RESULTS: The intra-class correlation coefficient (single measure) of the three raters was 0.806. The generated HSI was significantly correlated with the visual rating scales of the three raters (W.D.: 0.823, Q.L.: 0.817, C.S.: 0.717). HSI scores well differentiated the different HS categories as defined by the agreed HS visual rating (normal vs. mild: p < 0.001, mild vs. moderate: p < 0.001, moderate vs. severe: p = 0.001). CONCLUSIONS: The proposed HSI was consistent with visual rating scales from epileptologists and sensitive to HS severity. This MRI-based index may help to evaluate HS severity in clinical practice. Further validations are needed to associate HSI with post-surgery outcomes.

The interactome and proteomic responses of ALKBH7 in cell lines by in-depth proteomics analysis.

BACKGROUND: ALKBH7 is a mitochondrial protein, involved in programmed necrosis, fatty acid metabolism, cell cycle regulation, and prostate cancer disease. However, the exact roles of ALKBH7 and the underlying molecular mechanisms remain mysterious. Thus, investigations of the interactome and proteomic responses of ALKBH7 in cell lines using proteomics strategies are urgently required. METHODS: In the present study, we investigated the interactome of ALKBH7 in mitochondria through immunoprecipitation-mass spectrometry/mass spectrometry (IP-MS/MS). Additionally, we established the ALKBH7 knockdown and overexpression cell lines and further identified the differentially expressed proteins (DEPs) in these cell lines by TMT-based MS/MS. Two DEPs (UQCRH and HMGN1) were validated by western blotting analysis. RESULTS: Through bioinformatic analysis the proteomics data, we found that ALKBH7 was involved in protein homeostasis and cellular immunity, as well as cell proliferation, lipid metabolism, and programmed necrosis by regulating the expression of PTMA, PTMS, UQCRH, HMGN1, and HMGN2. Knockdown of ALKBH7 resulted in upregulation of UQCRH and HMGN1 expression, and the opposite pattern of expression was detected in ALKBH7 overexpression cell lines; these results were consistent with our proteomics data. CONCLUSION: Our findings indicate that the expression of UQCRH and HMGN1 is regulated by ALKBH7, which provides potential directions for future studies of ALKBH7. Furthermore, our results also provide comprehensive insights into the molecular mechanisms and pathways associated with ALKBH7.

Pallidal Deep Brain Stimulation in Patients With Chorea-Acanthocytosis.

INTRODUCTION: Chorea-acanthocytosis (ChAc) is an autosomal recessive hereditary disorder caused by the mutation of gene VPS13A. Deep brain stimulation of ChAc has made substantial progress in the recent decades. However, the reports were scattered across centers and performed by different neurosurgeons. Here, we report a case series consisting of six patients diagnosed with ChAc, receiving bilateral high-frequency stimulation of globus pallidus internus (GPi) in a single center. METHODS: We report six consecutive patients diagnosed with ChAc and present a review of the literature. All patients received neurological evaluations using the Unified Huntington's Disease Rating Scale (UHDRS) motor score before surgery and during clinical follow-ups. One patient was observed over six months, while five patients were seen over 12 months. RESULTS: All patients underwent high-frequency stimulation ranging from 130 Hz to 175 Hz. In the follow-up period, a general trend was found toward higher amplitude and broader pulse widths, with a mean current range of 2.08 mA to 3.06 mA and a mean pulse width range of 75 µsec to 98 µsec. On preoperative evaluation, the mean UHDRS motor score was 35.7 ± 16.3 and the chorea subscore was 11.3 ± 4.7. At the three-month postoperative follow-up, both UHDRS motor score (13.5 ± 5.8) and chorea subscore (3.0 ± 1.2) reached valley values. Thereafter, the UHDRS motor score and chorea subscore showed a gradual rise, reaching 19.2 ± 5.9 and 4.8 ± 1.7, respectively, at the 12-month follow-up. In addition, adverse events were also seen. Patient 1 developed dysarthria six months after surgery, whereas Patient 6 had a generalized tonic-clonic seizure attack one day after surgery CONCLUSION: High frequency stimulation of the GPi is an effective and safe modality for the treatment of ChAc, with both rapid symptomatic improvements and steady chronic outcomes.

Subthalamic Nuclei Stimulation in Patients With Pantothenate Kinase-Associated Neurodegeneration (PKAN).

INTRODUCTION: Pantothenate kinase-associated neurodegeneration (PKAN) is a rare autosomal recessive genetic disease that leads to extrapyramidal symptoms, such as dystonia, ataxia, dysarthria, and involuntary movements. Treatment of PKAN with deep brain stimulation (DBS) has been reported, but mainly focuses on targeting the globus pallidus internus (GPi). Subthalamic nuclei (STN) may also be a potential target for treatment of PKAN. METHODS: In this study, we reviewed three patients with PKAN (two with typical PKAN and one with atypical PKAN) treated by bilateral STN stimulation and present a review of the literature. All patients received neurological evaluation using the Burke-Fahn-Marsden Dystonia Rating Scale-movement (BFMDRS) scoring system before and after surgery. Patients were then subject to regular clinical follow-ups (ranging from 22 to 44 months). RESULTS: The mean stimulation amplitude, pulse width and frequency was 2.65 ± 0.45 V, 91.7 ± 21.9 µs, and 146.7 ± 12.5 Hz, respectively. BFMDRS scores were improved in all patients after surgery, ranging from 41.6 to 73.1%. Improvements of appendicular symptoms ranged from 46.2 to 94.1%, and improvements of axial symptoms ranged from 27.3 to 33.3%. No side effects were reported in patients 1 and 2; whereas patient 3 exhibited a mild decline in verbal fluency one year after surgery. CONCLUSION: STN stimulation could serve as a candidate DBS target in the treatment of PKAN, especially for patients with prominent appendicular symptoms.

Application of Preoperative CT/MRI Image Fusion in Target Positioning for Deep Brain Stimulation.

Objective To explore the efficacy of target positioning by preoperative CT/MRI image fusion technique in deep brain stimulation.Methods We retrospectively analyzed the clinical data and images of 79 cases (68 with Parkinson's disease, 11 with dystonia) who received preoperative CT/MRI image fusion in target positioning of subthalamic nucleus in deep brain stimulation. Deviation of implanted electrodes from the target nucleus of each patient were measured. Neurological evaluations of each patient before and after the treatment were performed and compared. Complications of the positioning and treatment were recorded.Results The mean deviations of the electrodes implanted on X, Y, and Z axis were 0.5 mm, 0.6 mm, and 0.6 mm, respectively. Postoperative neurologic evaluations scores of unified Parkinson's disease rating scale (UPDRS) for Parkinson's disease and Burke-Fahn-Marsden Dystonia Rating Scale (BFMDRS) for dystonia patients improved significantly compared to the preoperative scores (P<0.001); Complications occurred in 10.1% (8/79) patients, and main side effects were dysarthria and diplopia.Conclusion Target positioning by preoperative CT/MRI image fusion technique in deep brain stimulation has high accuracy and good clinical outcomes.

[Lateralizing value of dystonic posturing in patients with mesial temporal lobe epilepsy].

OBJECTIVE: To explore the lateralizing value of dystonic posturing (DP) of upper limb in patients with refractory mesial temporal lobe epilepsy (MTLE). METHODS: Presurgical videotypes of 89 patients staying seizure-free for at least 2 years after temporal lobectomy were retrospectively reviewed. Attention was paid to temporal correlation between occurrence of DP and seizure and the relationship of DP to side of epileptogenic zone (resected side). RESULTS: DP was observed in 92 complex partial seizure (CPS) from 37 (41.6%) patients among 89 patients with a total of 424 CPS. DP was not an initial symptom in the course of CPS and its onset occurred mostly in the middle third of ictus. DP displayed a high positive predictive value of 93.9% for lateralizing a contralateral seizure onset. CONCLUSION: DP is a reliable lateralizing sign in patients with MTLE.

Transsphenoidal approach for pituitary adenomas in patients with McCune-Albright syndrome.

The feasibility of transsphenoidal approach under a guidance of neuronavigation was explored to remove pituitary adenomas for patients with McCune-Albright syndrome (MAS). From August, 2008 to July, 2010, there were 5 patients diagnosed with MAS associated with a pituitary adenoma in our department of Peking Union Medical College Hospital. All the patients underwent transsphenoidal surgery for the removal of pituitary adenomas with the assistant of neuronavigation and all the procedures went uneventfully. Four of the five patients have got cured radiologically by imaging and 3 of them have got cured based on endocrinological criteria. Transsphenoidal approach under the neuronavigational guidance is a safe and effective management for the MAS patients with pituitary adenomas.

[Significance of pseudocapsule in the excision of pituitary adenomas in transsphenoidal surgery].

OBJECTIVE: To explore the significance of pseudocapsule in the excision of pituitary adenomas in transsphenoidal surgery. METHODS: For 22 patients with pituitary adenomas over a period of 2 years at Peking Union Medical College Hospital, resection of pseudocapsule was applied for complete tumor removal. Pituitary function test and radiological imaging were performed at pre-operation, 3 months post-operation and at subsequent 6-12 months intervals postoperatively. RESULTS: All pituitary adenomas were totally removed under microscope. The symptoms of headache, disorder of sight and visual field disappeared postoperatively in nonfunctional pituitary adenomas. The GH levels of 2/5 growth hormone secreting adenoma patients were 4.2 and 7.7 µg/L while it was under 1 µg/L for another 3. The postoperative level of prolactin was 4.3 µg/L in prolactin secreting adenoma. The level of adrenocorticotropic hormone decreased under 5 ng/L except one was 15.7 ng/L. Leakage of cerebrospinal fluid occurred intraoperatively in 3 patients and postoperatively in 1. No leakage was found after repair. Diabetes insipidus occurred in one patient and was controlled with Minirin. Pseudocapsule was confirmed by pathological examination. Special staining revealed reticulum fibers in pseudocapsule. CONCLUSION: Resection of pseudocapsule may achieve a higher remission rate without deteriorating pituitary function.

Study of the microstructure of brain white matter in medial temporal lobe epilepsy based on diffusion tensor imaging.

OBJECTIVES: To compare the white matter (WM) asymmetry in left and right medial temporal lobe epilepsy (mTLE) with and without hippocampal sclerosis (HS+, HS-) and assess the correlation of preoperative asymmetry and the dynamics of WM fibers with surgical outcomes. MATERIALS AND METHODS: Preoperative MRI scans were collected from 58 mTLE patients (40 HS+, 18 HS-); 15 (11 HS+, 4 HS-) then underwent postoperative MRI scans. DTI parameters, including the fractional anisotropy (FA), mean diffusion coefficient (MD), axial diffusion coefficient (AD), and radial diffusion coefficient (RD), were extracted from 20 paired WM tracts by PANDA based on the JHU WM tractography atlas. The bilateral cerebral parameters and the pre- to postoperative changes in the DTI parameters of specific fiber tracts were compared. The asymmetry indexes (AIs) of paired fibers were also analyzed. RESULTS: There were fewer asymmetrical WM fibers in HS- patients than in HS+ patients. The pattern of WM asymmetry differed between left and right mTLE patients. Differences in the FA AI of the inferior fronto-occipital fasciculus and inferior longitudinal fasciculus (ILF) were found in left HS+ patients with different surgical outcomes. All mTLE patients exhibited decreases in FA and increases in MD and RD in specific ipsilateral WM fibers. In International League Against Epilepsy (ILAE) grade 1 patients, the MD values in the ipsilateral CGH increased over time, whereas the RD values in the ipsilateral ILF and the AD values in the ipsilateral ILF and UNC decreased. In ILAE grade 2-5 patients, the FA values in the ipsilateral cingulate gyrus part of the cingulum (CGC) increased over time. CONCLUSION: The WM tract asymmetry was more extensive in HS+ patients than in HS- patients. The preoperative WM fiber AIs in left HS+ patients may be useful for surgical prognosis. Additionally, pre- to postoperative changes in WM fibers may help predict surgical outcomes.

An Independent Seizure-Onset Zone in Medial Temporal Lobe Found by 18 F-FDG PET Imaging Besides Epileptogenic Periventricular Nodular Heterotopia.

ABSTRACT: A 23-year-old man with drug-resistant epilepsy was admitted for presurgical evaluation. The epileptogenic zone could not be derived from seizure semiology and scalp electroencephalographic monitoring definitely. MRI showed periventricular nodular heterotopia in occipital horn of left lateral ventricle with high FDG uptake on interictal 18 F-FDG PET scan, whereas the hypometabolic zone in the left medial temporal lobe was also found on PET with no abnormality on MRI. Stereoelectroencephalographic implantation was performed to identify the seizure-onset zone. Two independent epileptogenic foci located in periventricular nodular heterotopia and left hippocampus were validated by stereoelectroencephalographic monitoring and the outcome of subsequent thermocoagulation.

A case of McCune-Albright syndrome associated with pituitary GH adenoma: therapeutic process and autopsy.

BACKGROUND: McCune-Albright syndrome (MAS) is a clinical syndrome with low incidence, and its concurrence with pituitary GH adenoma is rare. Little of the history, treatment and outcome has been studied. METHOD: Follow-up of a 37-year-old male patient of MAS associated with pituitary GH adenoma was performed continuously recording the disease development and the treatment process until death, after which an autopsy was performed. RESULTS: Radiation therapy (RT) efficaciously controlled GH hypersecretion, however, it may have been the cause of the malignant transformation of the dysplastic bone tissue, which eventually caused brain hernia and death; autopsy demonstrated that the cranium had significant thickening (as much as 10 cm), the pathological diagnosis was fibrous dysplasia of bone associated with chondrosarcoma; and undifferentiated chondrosarcoma with malignant fibrous histocytoma subtype in the sellar region; nodular goiter with the thyroid gland, one nodus was pathologically demonstrated as papillary carcinoma. CONCLUSION: GH adenoma, present in a patient with MAS, might be cured by RT; but the risk of malignant transformation of the dysplastic bone tissue in the field of irradiation make it controversial. Lessons from the case reported here told us that we should take great caution when recommending RT for patients like this.

[Clinical efficacy of microvascular decompression plus intraoperative monitoring of abnormal muscle response in the treatment of hemifacial spasm].

OBJECTIVE: To explore the clinical efficacy of microvascular decompression plus intraoperative monitoring of abnormal muscle response in the treatment of hemifacial spasm. METHODS: Between 2009 and 2010, a total of 47 patients underwent microvascular decompression for hemifacial spasm. There were 15 males and 32 females with an age range 23 - 70 years old. During operations, intermittent electrical pulses were applied to stimulate the zygomatic branch of facial nerve at the spasm side. And evoked potentials were monitored in orbicularis oris. All patients were followed up for 5 - 22 months. RESULTS: The abnormal muscle responses were recorded pre-operatively in all 47 patients at the spasm side. In 42 patients, the abnormal muscle responses disappeared at the different stages of operations (4 while opening dura, 9 while dissecting arachnoid membrane and 29 while separating responsible vessels). All 42 patients were cured during the follow-up period. In the remaining 5 patients, the abnormal muscle response were still recorded even at the end of operations. Two of 5 patients were free from spasm during the follow-up period while the symptoms of other 3 patients became obviously relieved. CONCLUSION: The combined approaches of microvascular decompression and intraoperative monitoring of abnormal muscle response may assist the identification of responsible vessels and improve the outcomes of hemifacial spasm.

[Clinical application of neuronavigation in transsphenoidal microsurgery of pituitary adenomas].

OBJECTIVES: To summarize the experiences in clinical application of neuronavigation in transsphenoidal microsurgery of specific pituitary adenomas, and to discuss its indications. METHODS: From January 2006 to December 2010, 138 cases of transsphenoidal microsurgery for specific pituitary adenomas under neuronavigation were reviewed. The indications for neuronavigation in transsphenoidal microsurgery includes: recurrent or regrowth of residual pituitary adenomas after former transsphenoidal surgery in 36 cases, invasive pituitary adenomas in 45 cases, extremely laterally or deeply situated microadenomas in 45 cases, poor pneumatization of the sphenoid in 4 cases, skull base anomalies due to osteodysplasia fibrosa in 3 cases, narrow space between bilateral internal carotid arteries in 4 cases, distortion of nasal septum in 1 case. RESULTS: In the recurrence group, 12 were totally removed, 9 subtotally removed; postoperative complications included hematoma within the tumor cavity in 2 cases, cerebrospinal fluid (CSF) leakage in 4 cases among which 3 developed intracranial infection and 2 communicating hydrocephalus, oculomotor paralysis in 1 case and hypopituitarism in 3 cases; 9 were cured and 8 remission. In the invasive group, 5 were totally removed, 27 subtotally removed; postoperative complications included hematoma within the tumor cavity in 1 case, CSF leakage and intracranial infection in 1 case; 2 were cured and 22 remission. None of the 30 invasive hormone-secreting adenomas were cured or remission. The 45 cases of hormone-secreting microadenomas were all totally removed, among which 38 were cured. Among the poor sphenoid pneumatization group, total and subtotal tumor removal were achieved in 2 cases respectively with only one cured. In the skull base anomaly group, 2 were totally removed and 1 subtotally removed, with only one cured. For the cases with narrow space between bilateral internal carotid arteries and distortion of nasal septum, all were totally removed and cured. CONCLUSIONS: Transsphenoidal microsurgery under neuronavigation can be applied for pituitary adenomas in above specific indications. It is an accurate, safe and effective approach for specific pituitary adenomas, which can not only expand the indication of transsphenoidal microsurgery for pituitary adenomas, but also reduce the harmful exposure of X-rays for the operating staff.

Proteomic changes in the hippocampus and motor cortex in a rat model of cerebral palsy: Effects of topical treatment.

Cerebral palsy (CP) is a non-progressive motor-impairment disorder related to brain injury early in development. To gain new insights into the mechanisms of CP and the therapeutic efficacy of Baimai ointment, we used a high-throughput quantitative proteomic approach to evaluate proteomic changes in the hippocampus and motor cortex in a rat model of CP induced by lipopolysaccharide (LPS) combined with hypoxia/ischemia (H/I). More than 2000 proteins were identified in each brain region with high confidence. Quantitative analysis demonstrated profound disturbances in the proteomes of the hippocampus and motor cortex after LPS + H/I, in addition to the disruption of the motor system. In contrast, the topical application of Baimai ointment not only alleviated the motor deficit in the CP model rats, but also restored the proteomes in the brain cortex. Furthermore, astrocytes in the hippocampus were strongly activated in the Baimai-treated CP rat brains, associated with an increase in neurotrophic factors. Proteomic analysis demonstrated that the CP model induced neuroinflammatory responses in the brain which were reversed by the topical application of Baimai ointment. This study highlights the unexpected roles of hippocampus and motor cortex neurons in CP progress and treatment, thus providing potentially novel therapeutic targets for CP.

Brain volume and perfusion asymmetry in temporal lobe epilepsy with and without hippocampal sclerosis.

Objectives: To detect and compare the features of interictal perfusion and volume asymmetry between temporal lobe epilepsy (TLE) patients with and without hippocampal sclerosis (HS).Methods: Sixty-one TLE patients (mean age 28.4 ± 9.3 years; 28 female/33 male) with unilateral signs of HS (TLE-HS+) and 25 TLE patients (mean age 29.8 ± 8.0 years; 17 female/8 male) without HS (TLE-HS-) were included. Thirty healthy volunteers served as controls (mean age 26.0 ± 8.7 years; 22 female/8 male). Brain segmentation and volume calculation were performed. Quantitative cerebral blood flow (CBF) values were measured based on arterial spin labeling (ASL). The asymmetry indices (AIs) of volume and perfusion were calculated.Results: TLE-HS+ (adjusted P = 0.001) and TLE-HS- patients (adjusted P = 0.006) had significantly higher hippocampal perfusion AIs than controls. TLE-HS+ and TLE-HS- had similar hippocampal perfusion AIs (adjusted P = 1.00). TLE-HS+ had higher hippocampal volume AIs than TLE-HS- and controls (adjusted P < 0.001). TLE-HS- and controls had similar hippocampal volume AIs (adjusted P = 1.00). All (100%) TLE-HS+ patients had positive hippocampal perfusion or volume AIs. No significant correlation between the AIs of hippocampal perfusion and volume was found in both TLE-HS+(P = 0.894) and TLE-HS- (P = 0.106) patients. TLE-HS+ patients demonstrated more extensive whole-brain asymmetry of both perfusion and volume than TLE-HS- patients.Conclusion: TLE-HS+ and TLE-HS- patients have different patterns of whole-brain perfusion and volume asymmetry. Hippocampal perfusion asymmetry was revealed in both TLE-HS+ and TLE-HS- patients.

Comparison of seizure outcomes and safety between anterior temporal lobotomy and lobectomy in patients with temporal lobe epilepsy.

Objectives: To compare the efficacy and safety of anterior temporal lobotomy (ATLo) and anterior temporal lobectomy (ATLe) in drug-resistant temporal lobe epilepsy.Methods: Patients diagnosed with pharmacoresistant temporal lobe epilepsy who underwent anterior temporal lobotomy (ATLo) or anterior temporal lobectomy (ATLe) performed by a single surgeon were retrospectively included. Every patient was followed up annually after surgery. The postoperative seizure outcome evaluation was based on the Engel and ILAE classifications. We compared postoperative complications and 2-year follow-up seizure outcomes between the ATLo group and the ATL group.Results: A total of 42 individuals (21 ATLo and 20 ATLe) were included. At the two-year follow-up, more patients in the ATLo group than the ATLe group had reached Engel class I (20 versus 14) and ILAE I (19 versus 13). However, these differences were not significant. One patient in the ATLo group had intraparenchymal hematoma and fully recovered. The two groups had similar incidences of other short-term complications, and no patients died or had any permanent complications.Discussion: ATLo is not inferior to ATLe for patients with drug-resistant temporal epilepsy. There was no significant difference in seizure outcomes or the rate of postoperative complications between the two groups. A large sample randomized control study is needed.

Proteomic profiling of sclerotic hippocampus revealed dysregulated packaging of vesicular neurotransmitters in temporal lobe epilepsy.

PURPOSE: Temporal lobe epilepsy (TLE) is the most common type of epilepsy. Hippocampal sclerosis is the most distinctive pathological feature of TLE; however, its role in the pathogenesis of TLE remains to be clarified. We performed global protein expression analysis of hippocampus from TLE patients and controls, aiming to reveal the molecular signaling pathways related to TLE. METHOD: Proteomic and bioinformatic analyses of the hippocampus were performed on 4 TLE and 4 control samples. High-resolution liquid chromatography coupled to tandem mass spectrometry (LC-MS/MS), in combination with TMT-6plex quantification, was applied for global protein expression analysis. The proteomics results were validated by Western blot with 25 TLE and 25 control individuals and Immunohistochemistry analysis with 33 TLE and 10 control individuals. RESULTS: Bioinformatics analysis demonstrated differentially expressed proteins in the synaptic vesicle pathway, the prostaglandin synthesis and regulation pathway and endocannabinoids and retrograde modulation of synaptic transmission pathway. Among these, excitatory amino acid transporter 1 (EAAT1) and Vesicular glutamate transporter 1 (VGLUT1) are critical for TLE and dysregulated expression might be closely related to the uptake of extracellular glutamate and contribute to the pathophysiology of TLE. Ras-related protein Rab-3A (RAB3A) downregulation might indicate the TLE-induced compensatory deficit in glutamate release. CONCLUSION: Our study indicates that expression of some proteins involved in the packaging of vesicular neurotransmitters is altered in TLE. In addition, upregulated expression of annexin family proteins, which are also related to TLE, might play an important role in protection against TLE.

Study of the hippocampal internal architecture in temporal lobe epilepsy using 7 T and 3 T MRI.

PURPOSE: To compare the hippocampal internal architecture (HIA) between 3 and 7 Tesla (T) magnetic resonance imaging (MRI) in patients with temporal lobe epilepsy (TLE), and to investigate the relationship between HIA and hippocampal volume, and postoperative outcomes. MATERIALS AND METHODS: Thirty-nine TLE patients were recruited with 3 and 7 T MRI scans and a semi-quantitative assessment of the HIA was performed. Differences in HIA scores between 3 and 7 T MRI were evaluated. HIA and hippocampal volume asymmetry were also calculated and compared. The utility of HIA and hippocampal volume asymmetry in epilepsy lateralization, and the predictive value between these two indicators were compared. The relationship between HIA and postoperative outcomes was investigated in 25 patients with amygdalohippocampectomy. RESULTS: HIA scores of epileptogenic hippocampi were lower than those of non-epileptogenic hippocampi at 3 and 7 T MRI. Higher HIA scores were observed at 7 T MRI. The HIA asymmetry and hippocampal volume asymmetry were both strong predictors for epilepsy lateralization and did not show difference in predictive value. No statistical differences in HIA asymmetry were observed between seizure-free patients (ILAE 1) compared to patients with seizures (ILAE 2-5). CONCLUSIONS: Visualization of hippocampal internal architecture (HIA) may be improved at 7 T MRI. HIA asymmetry is a significant predictor of laterality of seizure onset in TLE patients and has similar predictive value as hippocampal volume asymmetry, however, HIA asymmetry at 7 T does not have extra value in determining epilepsy lateralization and neither does predict surgical outcomes.

Type IIB focal cortical dysplasia with balloon cells in medial temporal lobe epilepsy: Clinical, neuroimaging, and histopathological findings.

PURPOSE: Type IIB focal cortical dysplasia (FCD) is an important cause of drug-resistant epilepsy. However, balloon cells located in the medial temporal lobe have been seldom reported. We aimed to discuss the clinical and pathological features of Type IIB FCD with balloon cells in the medial temporal lobe (MTLE-FCDIIB) and the differential diagnosis with other types of mesial temporal lobe epilepsy. METHODS: Three MTLE-FCDIIB cases were enrolled from Peking Union Medical College Hospital. Clinical and neuroimaging data were analyzed and histology features observed on hematoxylin-eosin (H&E) staining and immunochemical staining, including vimentin, nestin, S-100, CD34, neuronal nuclei antigen (Neun), glial fibrillary acidic protein (GFAP), neurofilament heavy chain (SMI32), were discussed. RESULTS: All cases involved drug-resistant epilepsy patients with childhood onset. The semiology of the epileptic seizure was a highly frequent partial seizure with or without generalized tonic-clonic seizures. Magnetic resonance imaging showed hyper-intensity in the medial temporal lobe without atrophy, different from mesial temporal sclerosis. Histological examination indicated the presence of balloon cells in the white matter of the para-hippocampal gyrus, subiculum, and cornu ammonis with cortical disorganization, and SMI32 positive dysmorphic neurons in the gray matter. Balloon cells were immunohistochemically stained with vimentin and nestin. Granular cell dispersion and pyramidal cell loss were not found. CONCLUSIONS: The presence of balloon cells in the medial temporal lobe is observed in a rare subgroup of FCD, named MTLE-FCDIIB. It has distinct clinical manifestations, neuroimaging features, pathological changes, and prognosis, which should be differentiated from mesial temporal lobe sclerosis and mesial temporal lobe tumors. Our findings enable more accurate diagnosis of mesial temporal lobe epilepsy.

Transsphenoidal surgery in a patient with acromegaly and McCune-Albright syndrome: application of neuronavigation.

The McCune-Albright syndrome (MAS) is characterized by a clinical triad of polyostotic fibrous dysplasia, café-au-lait hyperpigmented macules, and hypersecretory endocrinopathies. Acromegaly is an uncommon manifestation of the endocrine disturbance associated with MAS, and the role of surgery in managing these cases has been a topic of debate. The authors present the case of a 35-year-old man with MAS who was also diagnosed with acromegaly, hyperprolactinemia, and pituitary macroadenoma. The patient had an 18-year history of fibrous dysplasia involving the right frontal bone and ribs as well as multiple endocrinopathies, but no cutaneous hyperpigmented macules. An oral glucose tolerance test demonstrated partial suppression of plasma levels of growth hormone (GH). The patient underwent transsphenoidal resection of the pituitary tumor, performed with assistance of neuronavigation, and tolerated the procedure well. After the surgery, both prolactin and GH levels returned to normal. These results suggest that neuronavigation-assisted transsphenoidal surgery can safely remove pituitary adenomas associated with MAS and successfully treat the underlying endocrine abnormalities.