A cross-sectional study of people with epilepsy and neurocysticercosis in Tanzania: clinical characteristics and diagnostic approaches.

Neurocysticercosis (NCC) is a major cause of epilepsy in regions where pigs are free-ranging and hygiene is poor. Pork production is expected to increase in the next decade in sub-Saharan Africa, hence NCC will likely become more prevalent. In this study, people with epilepsy (PWE, n=212) were followed up 28.6 months after diagnosis of epilepsy. CT scans were performed, and serum and cerebrospinal fluid (CSF) of selected PWE were analysed. We compared the demographic data, clinical characteristics, and associated risk factors of PWE with and without NCC. PWE with NCC (n=35) were more likely to be older at first seizure (24.3 vs. 16.3 years, p=0.097), consumed more pork (97.1% vs. 73.6%, p=0.001), and were more often a member of the Iraqw tribe (94.3% vs. 67.8%, p=0.005) than PWE without NCC (n=177). PWE and NCC who were compliant with anti-epileptic medications had a significantly higher reduction of seizures (98.6% vs. 89.2%, p=0.046). Other characteristics such as gender, seizure frequency, compliance, past medical history, close contact with pigs, use of latrines and family history of seizures did not differ significantly between the two groups. The number of NCC lesions and active NCC lesions were significantly associated with a positive antibody result. The electroimmunotransfer blot, developed by the Centers for Disease Control and Prevention, was more sensitive than a commercial western blot, especially in PWE and cerebral calcifications. This is the first study to systematically compare the clinical characteristics of PWE due to NCC or other causes and to explore the utility of two different antibody tests for diagnosis of NCC in sub-Saharan Africa.

ADC histograms predict response to anti-angiogenic therapy in patients with recurrent high-grade glioma.

INTRODUCTION: The purpose of this study is to evaluate apparent diffusion coefficient (ADC) maps to distinguish anti-vascular and anti-tumor effects in the course of anti-angiogenic treatment of recurrent high-grade gliomas (rHGG) as compared to standard magnetic resonance imaging (MRI). METHODS: This retrospective study analyzed ADC maps from diffusion-weighted MRI in 14 rHGG patients during bevacizumab/irinotecan (B/I) therapy. Applying image segmentation, volumes of contrast-enhanced lesions in T1 sequences and of hyperintense T2 lesions (hT2) were calculated. hT2 were defined as regions of interest (ROI) and registered to corresponding ADC maps (hT2-ADC). Histograms were calculated from hT2-ADC ROIs. Thereafter, histogram asymmetry termed "skewness" was calculated and compared to progression-free survival (PFS) as defined by the Response Assessment Neuro-Oncology (RANO) Working Group criteria. RESULTS: At 8-12 weeks follow-up, seven (50%) patients showed a partial response, three (21.4%) patients were stable, and four (28.6%) patients progressed according to RANO criteria. hT2-ADC histograms demonstrated statistically significant changes in skewness in relation to PFS at 6 months. Patients with increasing skewness (n = 11) following B/I therapy had significantly shorter PFS than did patients with decreasing or stable skewness values (n = 3, median percentage change in skewness 54% versus -3%, p = 0.04). CONCLUSION: In rHGG patients, the change in ADC histogram skewness may be predictive for treatment response early in the course of anti-angiogenic therapy and more sensitive than treatment assessment based solely on RANO criteria.

Phenotypical variability of post-partum reversible cerebral vasoconstriction syndrome.

Reversible cerebral vasoconstriction syndrome is recognized increasingly as a complication of the postpartum period. Our series of four cases illustrates its phenotypical variability, summarizes the diagnostic work-up, and outlines potential treatment strategies for this usually benign but sometimes disabling and life-threatening disease.

Hypoplasia of deep cerebellar nuclei in joubert syndrome.

Abnormalities of deep cerebellar nuclei in Joubert syndrome have been previously reported only in rare autopsy cases. Epilepsy in association with Joubert syndrome is also rarely reported. In two new cases of patients with Joubert syndrome, bilateral hypoplasia of deep cerebellar nuclei was detected in vivo by magnetic resonance imaging. One of the patients had drug-resistant epilepsy. Both patients received clinical examination, electroencephalography, neuropsychologic testing, and high-resolution magnetic resonance imaging (1.5 T). Patient 1, a 7-year-old boy, had muscular hypotonia, periodic tachypnea, mild ataxia, global developmental delay, exotropia, and polydactyly. Patient 2, a 23-year-old woman, had muscular hypotonia, epilepsy with pharmacoresistant generalized tonic-clonic seizures, learning disability, esotropia, and mild gait ataxia. Abnormalities of deep cerebellar nuclei might contribute to the pathophysiology of epilepsy in patients with Joubert syndrome.

Asymmetric seizure termination in primary and secondary generalized tonic-clonic seizures.

PURPOSE: In temporal lobe epilepsies an asymmetric termination (AST) of the clonic phase of secondary generalized tonic-clonic seizures (sGTCS) reliably lateralizes the side of seizure onset. The last clonic activity occurs ipsilateral to the side of the seizure onset zone. We compared the prevalence and lateralizing value of AST in sGTCS of frontal and temporal lobe origin as well as in primary generalized tonic-clonic seizures (pGTCS). METHODS: We analyzed 177 seizures in 84 consecutive patients. Forty-one patients had temporal lobe epilepsy (TLE), 24 frontal lobe epilepsy (FLE), and 19 had nonfocal (primary) generalized epilepsies (GE). All patients underwent intensive video-EEG (electroencephalography) monitoring, high-resolution magnetic resonance imaging (MRI), neuropsychological testing, and single photon emission computed tomography/positron emission tomography (SPECT/PET) when feasible. Two investigators blinded for diagnosis, EEG, and imaging data assessed frequency and side of the last clonic jerk. RESULTS: AST occurred in 63% of patients with TLE (47% of seizures), in 71% with FLE (60% of seizures), and in 42% with GE (21% of seizures). These results were not significant for patients, but significant for seizures in TLE versus GE and in FLE versus GE (p < 0.001). The positive predictive value (PPV) for the side of seizure onset was 74% (p = 0.003) in TLE and 75% (p = 0.008) in FLE. DISCUSSION: AST in sGTCS lateralizes the side of seizure onset in TLE and in FLE to the ipsilateral hemisphere with a high PPV. However, AST was also observed in GE. Therefore, asymmetric clinical signs should not inevitably lead to the assumption of focal epilepsy syndromes.

Developmental trajectories of magnitude processing and interference control: an FMRI study.

Neurodevelopmental changes regarding interference and magnitude processing were assessed in 3 age groups (children, n = 10; young adults, n = 11; elderly participants, n = 9) by using an functional magnetic resonance imaging version of the numerical Stroop task. Behaviorally, comparable distance and size congruity effects were found in all 3 age groups. Distance effects were most pronounced in the more difficult numerical task, whereas size congruity effects were comparable across tasks. In response to interference, an age-linear trend in the pattern of activation in left and right prefrontal and left middle temporal regions of the brain was observed. This implicates that with increasing age interference control requires increasing effort (possible explanations for children's relatively lower interference effects are provided). In contrast, the distance effect produced a negative linear trend in right prefrontal, supplementary motor area, and intraparietal cortex. This suggests that relative to old adults, children and young adults had to recruit a larger network upon processing magnitude. The latter findings are even more remarkable considering that the behavioral effects were similar across groups. In summary, the developmental trajectories of interference control and magnitude processing differ, although these cognitive functions activate partially overlapping brain regions.

Epilepsy and neurocysticercosis in rural Tanzania-An imaging study.

PURPOSE: In developing countries, neurocysticercosis (NCC) is a common cause of epilepsy. Most of the work on NCC and epilepsy has been compiled in Latin America. To date, comprehensive neuroimaging studies are missing in sub-Saharan Africa. METHODS: In our study, we interviewed 212 people with epilepsy (PWE) and performed cerebral computed tomography (CT) at the Haydom Lutheran Hospital in northern Tanzania. Control cerebral CT scans were selected from 198 consecutive individuals without epilepsy. Sera of PWE with lesions indicating NCC (n = 20), PWE without NCC lesions (n = 20), and healthy individuals (n = 20), as well as cerebrospinal fluid (CSF) samples of PWE with NCC lesions (n = 11) were investigated for anticysticercal antibodies. RESULTS: Definite NCC lesions were present in five (2.4%), lesions highly suggestive of NCC in 24 (11.3%), and lesions compatible with NCC in nine (4.2%) PWE. This compares to two (1.0%) people with definite NCC lesions, two (1.0%) with lesions highly suggestive of, and six (2.9%) with lesions compatible with NCC in the control group. NCC lesions were significantly more frequent in PWE compared to controls (p < 0.0001). CT results, and serum and CSF analysis taken together, we diagnosed 22 (10.4%) individuals with probable and 7 (3.3%) with definitive NCC in our cohort of PWE. CONCLUSION: For the first time in sub-Saharan Africa, we give evidence within a large-scale neuroimaging study that NCC, a so far neglected infectious disease, represents a major cause of epilepsy.

An fMRI study of the numerical Stroop task in individuals with and without minimal cognitive impairment.

Aim of this functional magnetic resonance imaging (fMRI) study was to dissociate normal aging and minimal cognitive impairment (MCI) concerning magnitude processing and interference control. We examined the neural correlates of a numerical Stroop task in elderly individuals with and without MCI. Fifteen elderly participants (six patients with MCI and nine controls) were subjected to a numerical Stroop task requiring numerical/physical magnitude classifications while inhibiting task-irrelevant stimulus dimensions. Effects of distance and congruity were examined. Behaviourally, robust distance and congruity effects were observed in both groups and tasks. Imaging baseline conditions revealed stronger and more distributed activations in MCI patients relative to controls which could not be explained by the higher error rates committed by patients. Across tasks, conjunction analysis revealed highly significant activations in intra-parietal and prefrontal regions suggesting that both groups recruit comparable brain regions upon processing magnitude and interference, respectively. MCI patients exhibited stronger pre-/postcentral and thalamic activations, possibly reflecting more effortful response-selection processes or alternatively, deficient inhibitory control. Moreover, MCI patients exhibited additional activations in fronto-parietal (magnitude) and occipital/cerebellar (congruity) regions. To summarize, though MCI patients needed to recruit more distributed activation patterns conjunction analysis revealed common activation sites in response to magnitude processing and interference control.

Anticysticercal and antitoxocaral antibodies in people with epilepsy in rural Tanzania.

In developing countries, especially Latin America, neurocysticercosis (NCC) is a common cause of epilepsy. Recently, neurotoxocariasis has also been implicated in the pathogenesis of epilepsy. In sub-Saharan Africa data on parasitic disease and epilepsy are scarce. We therefore conducted a study in a rural hospital in northern Tanzania and analysed serum samples for anticysticercal and antitoxocaral antibodies for 40 people with epilepsy (PWE), 20 of whom had confirmed NCC on cranial computed tomography (CT) and 20 healthy individuals. Cerebrospinal fluid (CSF) of 11 PWE with NCC lesions on cranial CT was also investigated. Antibodies were determined using ELISA and Western blot. Six PWE with NCC lesions showed anticysticercal antibodies in serum. Of those, five had active lesions. Anticysticercal antibodies were significantly more frequent in PWE with active NCC than in those with inactive NCC (P<0.01). CSF samples were positive for anticysticercal antibodies in five patients, of whom four had active lesions on cranial CT. Antitoxocaral antibodies were detected in sera of 11 (55%) PWE with NCC lesions, of eight (40%) PWE without lesions on cranial CT and of eight (40%) controls. In our study anticysticercal antibodies in both serum and CSF were associated with active NCC in PWE, whereas there was no relationship between antitoxocaral antibodies and epilepsy.

The head nodding syndrome--clinical classification and possible causes.

PURPOSE: In the 1960s in Tanzania, L. Jilek-Aall observed a seizure disorder characterized by head nodding (HN). Decades later, "nodding disease," reminiscent of what was seen in Tanzania, was reported from Sudan. To date this seizure disorder has not been classified and possible causes still remain obscure. METHODS: In a prospective study in southern Tanzania, we evaluated 62 patients with HN. Selected patients underwent blood (n = 51) and cerebrospinal fluid (CSF) (n = 48) analyses. Others were chosen for MRI (n = 12) and EEG (n = 10). RESULTS: Seizure type was classified as "head nodding only" and "head nodding plus," the latter being combined with other types of seizure (n =34). During HN, consciousness was impaired in 11 patients (17.7%) and supportive signs of epileptic seizures were described by 15 (24.2%) patients. Precipitating factors were confirmed by 11 (17.7%) patients. Fifty-six (90.3%) patients had at least one relative with epilepsy. EEG confirmed interictal epileptic activity in two patients and unspecific changes in four patients. MRI showed hippocampus pathologies (n = 5) and gliotic changes (n = 5). Skin polymerase chain reaction (PCR) positivity for Onchocerca volvulus was significantly associated with lesions on MRI. However, PCR of the CSF was negative in all cases. CONCLUSIONS: We present a comprehensive clinical description of the "HN syndrome," possibly a new epilepsy disorder in sub-Saharan Africa. MRI lesions and their association with positive skin PCR for O. volvulus despite negative PCR of the CSF is intriguing and deserves attention. Furthermore, the high prevalence of hippocampus sclerosis and familial clustering of epilepsy may point toward other potential pathogenetic mechanisms.

Successful surgical treatment of insular epilepsy with nocturnal hypermotor seizures.

Nocturnal hypermotor seizures (NHSs) suggest seizure onset in the frontal lobe. We present a patient with NHSs and insular seizure onset who underwent successful surgical treatment. A 29-year-old right-handed man suffered from intractable NHSs since the age of 12 years. High-resolution MRI, [(18)F]FDG-PET, and neuropsychological examination gave normal results, ictal EEG was obscured by artifacts. Ictal [(99m)Tc]HMPAO-SPECT revealed hyperperfusion in the right anterior part of the insula and right frontal operculum. The seizure onset zone was localized in the right anterior insula based on invasive recordings. Electrical stimulation in that area elicited habitual seizures. A limited resection of the anterior part of the right insula and the right frontal operculum was performed rendering the patient seizure-free (follow-up 1 year). To our knowledge, this is the first reported nonlesional patient with an insular seizure onset and NHSs who underwent successful epilepsy surgery.

Tolerability of N-chlorotaurine in chronic rhinosinusitis applied via yamik catheter.

OBJECTIVES: A rational approach in the treatment of chronic rhinosinusitis (CRS) is the intranasal application of antiseptic agents, due to the pathogenetic role of bacteria and fungi. N-Chlorotaurine (NCT), a mild endogenous oxidant with broad-spectrum antimicrobial activity, has been tested for the first time in CRS. METHODS: This one-arm phase IIa clinical study is the first step in the clinical development of this promising substance for local therapy of CRS. The nasal and paranasal cavities of 12 patients were rinsed with 10-20 ml of 1% aqueous NCT solution, applied via a novel catheter system (YAMIK). Treatment consisted of three lavages per week for 4 weeks. RESULTS: NCT caused neither alterations of the mucosa nor burning pain during application. Nevertheless, the insertion of the catheter, the insufflation of the posterior cuff and the overpressure inside the sinuses after infiltration led to moderate pain in some patients. Mucosal swelling decreased in all subjects, nasal breathing could be improved in nine patients and impaired olfaction in seven. Polyps did not disappear within the 1-month period of the study. CONCLUSIONS: The good tolerability and possible beneficial effects of NCT encourage its further investigation in CRS. Despite some limitations the YAMIK catheter proved to be a convenient and safe device for rinsing the nasal and paranasal sinuses.

Rapidly progressive dysphagia caused by Forestier's disease: a case report.

Forestier's disease is a systemic rheumatologic abnormality of unknown etiology, characterized by a flowing ossification of the anterior ligament of the spine. In this case study we report on an atypical appearance of Forestier's disease in a 72-year-old woman. This patient had a one-month history of rapidly progressing dysphonia and dyspnea and at the time of admission was unable to eat. She had been operated for gastric and colon carcinoma seventeen months earlier. Total body CT scans showed a flowing ossification of the anterior ligament between levels C2 and C7 and an osteophyte protruding in the ventral direction at level C2. Before the osteophyte's removal, a tumor screening was conducted to exclude the presence of distant metastases or occult malignancy. Resection of the large osteophyte was performed via a typical ventral-cervical approach with horizontal skin incision. Six months after the procedure, the patient experienced distinct improvement of her condition and was able to eat both pulpy and solid foods again. In this case example, a rapidly progressive dysphagia was not caused by secondary malignancy, as initially speculated, but resulted from a ventral-cervical osteophyte, considered a rare cause for acute development of severe dysphagia. Early diagnosis of Forestier's disease in this patient led to immediate surgical intervention, which proved to be adequate treatment and enabled the patient to make a good recovery.

Paranasal and orbital anatomy revisited: identification of the ethmoid arteries on coronal CT scans.

OBJECTIVE: The aim of this study was to identify recognizable landmarks on coronal CT (CCT) scans for the localization of the anterior and posterior ethmoid arteries, which are important anatomic structures and surgical landmarks within the ethmoid sinuses. MATERIALS AND METHODS: Four series of plastinated cadaver specimens and 80 CCT examinations were evaluated to identify the anatomical landmarks that define the course of the anterior and posterior ethmoid arteries within the ethmoid sinuses. RESULTS: The following anatomic landmarks: the indentations into the medial orbital wall, the relationship between the superior oblique and medial rectus orbital muscles, and the lateral ethmoid fovea wall thinning proved to be useful CCT landmarks for the localization of the anterior and posterior ethmoid arteries. CONCLUSION: The results of this study suggest that familiarity with the above-mentioned landmarks on routine CCT images facilitates the localization of the anterior and posterior ethmoid arteries as they enter the ethmoid sinus.

Appendicitis: should diagnostic imaging be performed if the clinical presentation is highly suggestive of the disease?

BACKGROUND & AIMS: Our aim was to investigate whether diagnostic imaging is required if the clinical presentation suggests acute appendicitis with high probability. METHODS: On the basis of clinical findings, 350 consecutive patients with clinical suspicion of acute appendicitis were prospectively divided into 3 groups as follows: low, intermediate, and high probability of having appendicitis. All patients then underwent diagnostic ultrasonography. The clinical likelihood of appendicitis and the ultrasonographic results were correlated with the definite diagnoses. RESULTS: In the patients with clinically low probability of having appendicitis, appendicitis was present in 10% (11 of 109 patients), and, in those with intermediate probability, appendicitis was present in 24% (23 of 97 patients). Patients with clinically high probability of having appendicitis had appendicitis in 65% (94 of 144 patients), an alternative diagnosis in 18% (26 of 144 patients), and no specific definitive diagnosis in 17% (24 of 144 patients). Ultrasonography diagnosed appendicitis and the differential diagnoses with a sensitivity of 98% and 97%, specificity of 98% and 100%, positive predictive value of 96% and 99%, negative predictive values of 99% and 99%, and accuracy of 98% and 99%, respectively. CONCLUSIONS: Even in patients with clinically high probability of acute appendicitis, diagnostic imaging should be performed because it accurately depicts a high percentage of normal appendices and differential diagnoses.