Mononeuritis multiplex: association with infectious condition and familial background in a tropical environment: a case report.

Mononeuritis multiplex is characterized by an asymmetric pattern with affection of the peripheral nervous system; this form of polyneuropathy is often seen in non-systemic vasculitis. We present a case of multiplex neuropathy in a patient with histologicaly verified Hailey-Hailey disease. With the exception of this comorbidity--in its characteristic form presenting additionally with a superinfected subdermal node--we did not find any other possible etiologic factor possibly causative of multiplex neuritis. The diagnosis was confirmed by electrophysiological testing. To our knowledge, this is the first case report indicating a possible relationship between Hailey-Hailey disease and multiplex neuritis. There exists only one related study in the literature, which was conducted in Columbia--our patient's home country. This study delineates a clinically similar dermal disease (pemphigus foliaceus) in patients from rural Colombia (El Bagre). The authors detected anti-neuronal antibodies which were interpreted to be responsible for the pathognomonic burning sensations.

Neurological disorders.

Neurological conditions during pregnancy can be pregnancy related or can be caused by exacerbation of pre-existing neurological disorders. Knowledge of pre-existing epilepsy or myasthenia gravis in women of childbearing age requires preconception counselling by neurologist and planned pregnancy. Possible adverse effects of medication on the foetus should be balanced with the risk of uncontrolled symptoms. Interdisciplinary management before, during and after pregnancy is recommended. New acute neurological symptoms in pregnant or postpartum women should lead to an urgent neurological review. Patients need a thorough diagnostic evaluation that targets a range of serious pathological conditions that are either unique to (e.g. eclampsia) or arise more frequently (e.g. cerebral venous thrombosis) in this population. Most of these conditions are infrequent and require a specialized and multidisciplinary management. Treatment is challenging due to risks to the unborn child.

Epilepsy: recent advances.

This short review summarizes epilepsy papers published in the Journal of Neurology in 2009, covering pathophysiology (inflammation; propagation pathways), new treatments (oxcarbazepine in paediatrics; levetiracetam in status epilepticus), and non-seizure outcomes (violence; foetal/pregnancy).

Limbic system abnormalities associated with Ammon's horn sclerosis do not alter seizure outcome after amygdalohippocampectomy.

PURPOSE: To evaluate whether limbic system abnormalities associated with Ammon's horn sclerosis alter seizure outcome after selective amgydalohippocampectomy. METHODS: In 45 patients with unilateral mesial temporal lobe epilepsy, histologically proven Ammon's horn sclerosis, and uneventful postoperative course, volumes of the hippocampus, hemisphere, amygdala, entorhinal cortex, mamillary body, and fornix were measured by using a T(1)-weighted 3-D gradient-echo sequence with roughly isotropic (1.17 x 1.17 x 1-mm) voxels. In addition, signal intensity of the hippocampus and of the temporal lobe white matter was visually assessed and graded on a coronal T(2)-weighted fast-spin-echo sequence with 2-mm-thick slices. Volumetric measurements and visual analysis were compared between seizure-free and non-seizure-free patients examined 12 months after surgery. RESULTS: Hippocampal, hemispheric, entorhinal cortex, mamillary body, and fornix volumes, but not amygdalar volumes, were significantly smaller on the operated-on than on the non-operated-on side and significantly smaller in patients compared with controls. No volume differences of the hippocampus, hemisphere, amygdala, entorhinal cortex, mamillary body, and fornix existed between seizure-free (Engel class IA) and non-seizure-free patients (Engel class IB-IV). Increased temporal lobe white matter signal was observed in 15 patients but did not alter seizure outcome. CONCLUSIONS: Limbic system abnormalities are not a surrogate marker to predict postsurgical seizure outcome in patients with unilateral Ammon's horn sclerosis.

MR imaging in the presurgical workup of patients with drug-resistant epilepsy.

BACKGROUND AND PURPOSE: Whether an epileptic lesion is detected with MR imaging depends on the quality of the images and the expertise of the reader. We analyzed the role of 1.5-T MR imaging in the presurgical evaluation of patients with drug-resistant epilepsy at one center. METHODS: In a 2-year prospective study, 385 patients with drug-resistant epilepsy underwent standardized MR imaging at 1.5 T. We analyzed whether lesions were detected, whether they were precisely characterized by MR imaging, and whether lesion characterization allowed us to estimate seizure outcomes. RESULTS: Lesions were found on MR images in 318 patients (83%). Following presurgical evaluation, 209 (66%) underwent surgery, and 109 (34%) did not. Freedom from seizures was achieved in 130 (70%) of 186 patients. Nine (14%) of 66 patients without an MR imaging lesion underwent surgery; histopathologic findings were unrevealing in seven patients, and five (56%) achieved freedom from seizures. Hippocampal sclerosis was the most common lesion (52%) and correctly characterized in 101 (97%) of 104 patients. Glioneuronal tumors (20%) were sometimes imprecisely characterized: Four nonenhancing gangliogliomas were mistaken for focal cortical dysplasias. Outcomes were not different between lesion groups. However, there were trends toward a favorable outcome for focal cortical dysplasias with balloon cells and an unfavorable outcome for gyral scars. CONCLUSION: MR imaging detection of lesions influences further presurgical workup, though lesion characterization does not allow us to predict seizure outcome. If MR imaging fails to depict a lesion and patients undergo surgery because of electrophysiologic findings, histopathologic findings are often unrevealing.

Neuronal substrates of sensory gating within the human brain.

BACKGROUND: For the human brain, habituation to irrelevant sensory input is an important function whose failure is associated with behavioral disturbances. Sensory gating can be studied by recording the brain's electrical responses to repeated clicks: the P50 potential is normally reduced to the second of two paired clicks but not in schizophrenia patients. To identify its neural correlates, we recorded electrical traces of sensory gating directly from the human hippocampus and neocortex. METHODS: Intracranial evoked potentials were recorded using hippocampal depth electrodes and subdural strip and grid electrodes in 32 epilepsy patients undergoing invasive presurgical evaluation. RESULTS: We found evidence of sensory gating only in the hippocampus, the temporo-parietal region (Brodmann's areas 22 and 2), and the prefrontal cortex (Brodmann's areas 6 and 24); however, whereas neocortical habituating responses to paired clicks were peaking around 50 msec, responses within the hippocampus proper had a latency of about 250 msec. CONCLUSIONS: Consistent with data from animal studies, our findings show that the hippocampus proper contributes to sensory gating, albeit during a time window following neocortical habituation processes. Thus, sensory gating may be a multistep process, with an early phase subserved by the temporo-parietal and prefrontal cortex and a later phase mediated by the hippocampus.

Correlation of hippocampal glucose oxidation capacity and interictal FDG-PET in temporal lobe epilepsy.

PURPOSE: Interictal [18F]fluorodeoxyglucose (FDG) positron emission tomography (PET) demonstrates temporal hypometabolism in the epileptogenic zone of 60-90% of patients with temporal lobe epilepsy. The pathophysiology of this finding is still unknown. Several studies failed to show a correlation between hippocampal FDG-PET hypometabolism and neuronal cell loss. Because FDG is metabolized by hexokinase bound to the outer mitochondrial membrane, we correlated the glucose-oxidation capacity of hippocampal subfields obtained after surgical resection with the corresponding hippocampal presurgical FDG-PET activity. METHODS: In 16 patients with electrophysiologically confirmed temporal lobe epilepsy, we used high-resolution respirometry to determine the basal and maximal glucose-oxidation rates in 400-microm-thick hippocampal subfields obtained after dissection of human hippocampal slices into the CA1 and CA3 pyramidal subfields and the dentate gyrus. RESULTS: We observed a correlation of the FDG-PET activity with the maximal glucose-oxidation rate of the CA3 pyramidal subfields (rp = 0.7, p = 0.003) but not for the regions CA1 and dentate gyrus. In accordance with previous studies, no correlation of the FDG-PET to the neuronal cell density of CA1, CA3, and dentate gyrus was found. CONCLUSIONS: The interictal hippocampal FDG-PET hypometabolism in patients with temporal lobe epilepsy is correlated to the glucose-oxidation capacity of the CA3 hippocampal subfield as result of impaired oxidative metabolism.

Digital photography and 3D MRI-based multimodal imaging for individualized planning of resective neocortical epilepsy surgery.

PURPOSE: Invasive presurgical work up of pharmacoresistant epilepsies presumes integration of multiple diagnostic modalities into a comprehensive picture of seizure onset and eloquent brain areas. During resection, reliable transfer of evaluation results to the patient's individual anatomy must be made. We investigated the value of digital photography-based grid localization in combination with preoperative three-dimensional (3D) magnetic resonance imaging (MRI) for clinical routine. METHODS: Digital photographs of the exposed cortex were taken before and after grid placement. Location of electrode contacts on the cortex was identified and schematically indicated on native cortex prints. Accordingly, transfer of contact positions to a 3D MRI brain-surface rendering was carried out manually by using the rendering software. Results of the electrophysiologic evaluation were transferred to either electrode contact reproduction and co-registered with imaging-based techniques such as single-photon emission computed tomography (SPECT), positron emission tomography (PET), and functional MRI (fMRI). RESULTS: Digital photography allows precise and highly realistic documentation of electrode contact positions on the individual neocortical surface. Lesions underneath grids can be highlighted by semitransparent MRI surface rendering, and lobar boundaries can be identified. Because of integrating electrode contact positions into the postprocessed 3D MRI data set, imaging-based techniques can be codisplayed with the results of the electrophysiologic evaluation. Comparison with CT/MRI co-registration showed good accuracy of the method. However, grids not sewn to the dura at implantation can become subject to significant displacement. CONCLUSIONS: Digital photography in combination with preimplantation 3D MRI allows the generation of reliable tailored resection plans in neocortical epilepsy surgery. The method enhances surgical safety and confidence.

Selective middle cerebral artery Wada tests as a part of presurgical evaluation in patients with drug-resistant epilepsies.

PURPOSE: To analyze the role of selective middle cerebral artery (MCA) Wada tests in the presurgical workup of patients with drug-resistant focal epilepsies. METHODS: Twenty MCA Wada test procedures were performed to identify eloquent cortex (a) in nine patients with hemispheric lesions involving the motor cortex (connatal MCA infarct, n = 5; unilateral cortical dysplasia, n = 3; Rasmussen encephalitis, n = 1), (b) five patients with circumscribed neoplastic or nonneoplastic lesions adjacent to the motor cortex or classic language areas, and (c) for purely electrophysiologic reasons, in two patients with electrical status epilepticus in sleep (ESES). Sodium amobarbital and [99mTc]-HMPAO were simultaneously injected via a microcatheter into the distal M1 segment (n = 7), the inferior MCA trunk (n = 3), or into MCA branches (n = 10). RESULTS: Co-registered single-photon emission computed tomography (SPECT) and magnetic resonance imaging (MRI;n = 18) showed that sodium amobarbital did not reach the target area in three procedures. Temporary neurologic deficits occurred in 12 procedures. Eleven patients were operated on with the following surgical approaches: functional hemispherectomy, n = 3; partial or extended lesionectomy, n = 4; anterior temporal lobectomy, n = 1; and multiple subpial transsections, n = 3. Seizure freedom (Engel class I) was achieved in five patients. CONCLUSIONS: Selective MCA Wada tests can contribute to risk assessment concerning postsurgical motor deficits before functional hemispherectomy. Other indications are less clear: The identification of language areas is targeted primarily by electrical-stimulation mapping after subdural grid implantations, and selective MCA Wada tests in ESES patients yet have to be validated in larger patient groups.

Focal cortical dysplasia of Taylor's balloon cell type: a clinicopathological entity with characteristic neuroimaging and histopathological features, and favorable postsurgical outcome.

BACKGROUND AND PURPOSE: Focal cortical dysplasia of Taylor's balloon-cell type (FCD-BC) are a frequent cause of pharmacoresistant epilepsy in young patients. In order to characterize FCD-BC, we coupled MRI and histopathology, and analyzed the clinical outcome following epilepsy surgery. METHODS: From an epilepsy data bank with 547 histological specimens, 17 FCD-BC were re-evaluated of which high resolution MRI was available. Five additional FCD-BC were prospectively identified by MRI. Histopathological and immunohistochemical features were related to MRI. Outcome following lesionectomy was analyzed as determined on routine examinations 3, 6 and 12 months following surgery. RESULTS: All but one lesion were located outside the temporal lobe. A markedly hyperintense funnel-shaped subcortical zone tapering towards the lateral ventricle was the characteristic finding on FLAIR MRI. Histopathologically, the subcortical zone of the FCD-BC displayed hypomyelinated white matter with radially oriented balloon cells and gliosis. Dysplastic neurons were found in the adjacent, disorganized cortex. All patients with complete lesionectomy were seizure free one year following surgery. CONCLUSION: Focal cortical dysplasias of Taylor's balloon-cell type (FCD-BC) have characteristic MRI and histopathological findings. MRI recognition is important, since outcome following resective surgery is favorable.