The Sotos syndrome is an autosomal dominant disorder characterized by haploinsufficiency of
gene, with some individuals affected by epilepsy and, rarely, drug-resistant seizures. A 47-years-old ...female patient with Sotos syndrome was diagnosed with focal-onset seizures in left temporal lobe, left-side hippocampal atrophy, and neuropsychological testing with decreased performance in several cognitive domains. Patient was treated with left-side temporal lobe resection and developed complete awake seizure control in 3-years of follow-up, with marked improvement in quality-of-life. In selected, clinically concordant patients, resective surgeries may play a significant role in improving patient's quality of life and seizure control.
OBJECTIVE The authors report a novel surgical route from a superior anatomical aspect-the contralateral anterior interhemispheric-transcallosal-transrostral approach-to a lesion located in the ...subcallosal region. The neurosurgical approach to the subcallosal region is challenging due to its deep location and close relationship with important vascular structures. Anterior and inferior routes to the subcallosal region have been described but risk damaging the branches of the anterior cerebral artery. METHODS Three formalin-fixed and silicone-injected adult cadaveric heads were studied to demonstrate the relationships between the transventricular surgical approach and the subcallosal region. The surgical, clinical, and radiological history of a 39-year-old man with a subcallosal cavernous malformation was retrospectively used to document the neurological examination and radiographic parameters of such a case. RESULTS The contralateral anterior interhemispheric-transcallosal-transrostral approach provides access to the subcallosal area that also includes the inferior portion of the pericallosal cistern, lamina terminalis cistern, the paraterminal and paraolfactory gyri, and the anterior surface of the optic chiasm. The approach avoids the neurocritical perforating branches of the anterior communicating artery. CONCLUSIONS The contralateral anterior interhemispheric-transcallosal-transrostral approach may be an alternative route to subcallosal area lesions, with less risk to the branches of the anterior cerebral artery, particularly the anterior communicating artery perforators.
•DBS of the subthalamic nucleus improves quality of life.•DBS of the subthalamic nucleus reduces levodopa equivalent doses.•DBS of the subthalamic nucleus improves motor functions in both on and ...off-medication periods.
This study analyzed the influence of subthalamic nucleus deep brain stimulation (DBS) in motor parameters and patients' quality of life with Parkinson's disease (PD) evaluated before and after 12 months of the surgical procedure.
A cohort of 20 patients with PD who underwent DBS implantation in the subthalamic nucleus was included. Pre and on-DBS postoperative data in on and off-medication periods related to motor functions and quality of life, from the application of validated scales, were collected to verify possible relationships between changes in these parameters and the surgical procedure.
A significant decrease in the Hoehn and Yahr scale disease stage and in the levodopa equivalent dose (p < 0.001) was verified in the off-medication period when we compared baseline and post-12 months data. A significant decrease in dyskinesias (p = 0.009) was observed during the on-medication period by evaluating the UDysRS scale. Concerning motor functions verified through the UPDRS-III scale, it was obtained a significant reduction in total scores (p = 0.001), besides a decrease in rigidity scores for upper and lower limbs (p < 0.05) during the on-medication period. During the off-medication period, scores of UPDRS-III demonstrated a significant decrease, except for the ones related to speech and amplitude of resting tremor. Regarding the quality-of-life assessment, scores obtained from PDQ-39 showed that, after 12 months of electrode implantation, there was a significant decrease in mobility, daily living activity, and stigma parameters (p < 0.05).
Results obtained allow us to conclude that DBS of the subthalamic nucleus in patients with PD improves motor function in both on and off-medication periods, improvement of the disease stage in the off-medication period, with no change in the on-medication period, also an improvement in the patient's self-reported quality of life and a significant reduction in the dose of L-DOPA.
Mesial temporal lobe epilepsy (MTLE) is usually associated with drug-resistant seizures and cognitive deficits. Efforts have been made to improve the understanding of the pathophysiology of MTLE for ...new therapies. In this study, we used proteomics to determine the differential expression of proteins in the hippocampus of patients with MTLE compared to control samples. By using the two-dimensional electrophoresis method (2-DE), the proteins were separated into spots and analyzed by LC-MS/MS. Spots that had different densitometric values for patients and controls were selected for the study. The following proteins were found to be up-regulated in patients: isoform 1 of serum albumin (ALB), proton ATPase catalytic subunit A (ATP6V1A), heat shock protein 70 (HSP70), dihydropyrimidinase-related protein 2 (DPYSL2), isoform 1 of myelin basic protein (MBP), and dihydrolipoamide S-acethyltransferase (DLAT). The protein isoform 3 of the spectrin alpha chain (SPTAN1) was down-regulated while glutathione S-transferase P (GSTP1) and protein DJ-1 (PARK7) were found only in the hippocampus of patients with MTLE. Interactome analysis of the nine proteins of interest revealed interactions with 20 other proteins, most of them involved with metabolic processes (37%), presenting catalytic activity (37%) and working as hydrolyses (25%), among others. Our results provide evidence supporting a direct link between synaptic plasticity, metabolic disturbance, oxidative stress with mitochondrial damage, the disruption of the blood⁻brain barrier and changes in CNS structural proteins with cell death and epileptogenesis in MTLE. Besides this, the presence of markers of cell survival indicated a compensatory mechanism. The over-expression of GSTP1 in MTLE could be related to drug-resistance.
Highlights • Performing amygdalohippocampectomy without temporal lobe damage and preserving neurological functions maximally is crucial. • Better anatomical knowledge is necessary for surgical ...procedure of temporal epilepsy. • We aimed to combine our clinical experiences and anatomic trick points of transcortical selective amygdalohippocampectomy.
Brain edema is the leading cause of death in patients with malignant middle cerebral artery (MCA) infarction. Midline shift (MLS) has been used as a monohemispheric brain edema marker in several ...studies; however, it does not precisely measure brain edema. It is now possible to directly measure hemisphere brain volume. Knowledge about the time course of brain edema after malignant middle cerebral artery infarction may contribute to the condition's management.
Therefore, our goal was to evaluate the course of brain edema in patients with malignant MCA infarction treated with decompressive craniectomy (DC) using hemispheric volumetric measurements.
Patients were selected consecutively from a single tertiary hospital between 2013 and 2019. All patients were diagnosed with malignant middle cerebral artery infarction and underwent a decompressive craniectomy (DC) to treat the ischemic event. All computed tomography (CT) exams performed during the clinical care of these patients were analyzed, and the whole ischemic hemisphere volume was calculated for each CT scan.
We analyzed 43 patients (197 CT exams). Patients' mean age at DC was 51.72 range: 42-68 years. The mean time between the ischemic ictus and DC was 41.88 (range: 6-77) hours. The mean time between the ischemic event and the peak of hemisphere volume was 168.84 (95% confidence interval 142.08, 195.59) hours.
In conclusion, the peak of cerebral edema in malignant MCA infarction after DC occurred on the 7th day (168.84 h) after stroke symptoms onset. Further studies evaluating therapies for brain edema even after DC should be investigated.
The objective of the study was to analyze preoperative visual and verbal episodic memories in a homogeneous series of patients with mesial temporal lobe epilepsy (MTLE) and unilateral hippocampal ...sclerosis (HS) submitted to corticoamygdalohippocampectomy and its association with neuronal cell density of each hippocampal subfield.
The hippocampi of 72 right-handed patients were collected and prepared for histopathological examination. Hippocampal sclerosis patterns were determined, and neuronal cell density was calculated. Preoperatively, two verbal and two visual memory tests (immediate and delayed recalls) were applied, and patients were divided into two groups, left and right MTLE (36/36).
There were no statistical differences between groups regarding demographic and clinical data. Cornu Ammonis 4 (CA4) neuronal density was significantly lower in the right hippocampus compared with the left (p=0.048). The groups with HS presented different memory performance — the right HS were worse in visual memory test Complex Rey Figure, immediate (p=0.001) and delayed (p=0.009), but better in one verbal task RAVLT delayed (p=0.005). Multiple regression analysis suggested that the verbal memory performance of the group with left HS was explained by CA1 neuronal density since both tasks were significantly influenced by CA1 Logical Memory immediate recall (p=0.050) and Logical Memory and RAVLT delayed recalls (p=0.004 and p=0.001, respectively). For patients with right HS, both CA1 subfield integrity (p=0.006) and epilepsy duration (p=0.012) explained Complex Rey Figure immediate recall performance. Ultimately, epilepsy duration also explained the performance in the Complex Rey Figure delayed recall (p<0.001).
Cornu Ammonis 1 (CA1) hippocampal subfield was related to immediate and delayed recalls of verbal memory tests in left HS, while CA1 and epilepsy duration were associated with visual memory performance in patients with right HS.
•Patients with right HS showed less neuronal cell density in CA4.•Verbal memory is related with CA1 in those with left HS.•Visual memory is related with CA1 and epilepsy duration in right HS.
Summary Psychiatric outcomes of patients submitted to epilepsy surgery have gained particular interest given the high prevalence of pre-surgical psychiatric disorders (PD) in this population. The ...present study aimed to verify the possible pre-surgical predictors for psychiatric disorders following epilepsy surgery in a homogeneous series of patients with refractory temporal lobe epilepsy and mesial temporal sclerosis (TLE-MTS). Data from 115 TLE-MTS patients (65 females; 56.5%) who underwent cortico-amygdalohippocampectomy were included. Pre- and post-surgical psychiatric evaluations were performed using DSM-IV criteria. Pre-surgical PD – particularly mood, anxiety and psychotic disorders – were diagnosed in 47 patients (40.8%). Twenty-seven patients (54% of those with pre-surgical PD) demonstrated a remission of psychiatric symptoms on post-surgical psychiatric evaluation. Eleven patients (9.6%) developed de novo PD. The presence of pre-surgical depression (OR = 3.32; p = 0.008), pre-surgical interictal psychosis (OR = 4.39; p = 0.009) and epileptiform discharges contralateral to the epileptogenic zone (OR = 2.73; p = 0.01) were risk factors associated with post-surgical PD. Although epilepsy surgery is considered to be the best treatment option for patients with refractory TLE-MTS, the relatively high psychiatric comorbidities observed in surgical candidates and their possible negative impact on post-surgical outcomes require a careful pre-surgical evaluation of clinical, sociodemographic and psychiatric factors.
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