Headaches More Common among Epilepsy Sufferers with Neurocysticercosis than Other Structural Brain Lesions.

Neurocysticercosis is a leading cause of seizures and epilepsy in the developing world. Cysticercosis is endemic in many regions of Central and South America, sub-Saharan Africa, India, and Asia. Neurocysticercosis is of emerging importance because globalization has increased travel between Hawai'i and disease-endemic areas. Headache and epilepsy are two of the most common complications of neurocysticercosis infection. Currently, it is not known if epilepsy patients with neurocysticercosis are more likely to have headaches than those with other structural brain lesions or those with no structural brain abnormalities. This study was designed to investigate whether epilepsy patients with neurocysticercosis report co-morbid headaches more frequently than those with other or with no structural brain lesions. A retrospective cross-sectional study of all patients treated at a community based neurology clinic for epilepsy during a three-month period was performed. One-hundred sixty patients were included in the analytical study. Co-morbid headaches were more commonly present among those with neurocysticercosis (40%) than those with other structural lesions and those with no structural brain abnormalities (19% and 22%, respectively; P = .031). Headache frequency among those reporting co-morbid headaches did not differ significantly between the groups. Prevalence of co-morbid headaches is greater among epilepsy patients with neurocysticercosis than those with other structural brain lesions or no structural brain abnormality. Epilepsy patients with neurocysticercosis may be especially vulnerable to development of headaches and a thorough headache history should be obtained to help screen for affected individuals.

α7-Nicotinic acetylcholine receptor inhibition by indinavir: implications for cognitive dysfunction in treated HIV disease.

OBJECTIVE: The study set out to determine if the HIV protease inhibitor, indinavir, alters responsiveness of α7-nicotinic acetylcholine receptors to acetylcholine. DESIGN: Treatment with HAART has dramatically reduced development of HIV-associated dementia and more severe forms of cognitive impairment. However, many individuals continue to experience cognitive decline of uncertain cause. Previous studies have failed to demonstrate significant alterations of functional brain connectivity, structural brain changes, or changes in cerebral blood flow sufficient to explain cognitive decline in virally suppressed individuals. This suggests that the mechanisms underlying development and progression of cognitive problems likely occurs at a micro rather than macro level, such as disruptions in neurotransmitter system signaling. MATERIALS AND METHODS: Indinavir's effects on α7-nicotinic acetylcholine receptor activity was tested using a ScreenPatch IonWorks Barracuda-based assay in a mammalian cell model. RESULTS: At low concentrations (0.0003-10 µmol/l) indinavir acts as a positive allosteric modulator (EC50 = 0.021 µmol/l), whereas at concentrations greater than 10 µmol/l (30-100 µmol/l) indinavir acts as an inhibitor of the α7-nicotinic acetylcholine receptor. CONCLUSION: At concentrations greater than 10 µmol/l indinavir reduces synaptic transmission in the acetylcholine neurotransmitter system, which could possibly contribute to cognitive dysfunction. These results suggest that further experiments should be considered to assess whether patients might benefit from treatment with cholinesterase inhibitors that counteract the effects of indinavir.

CNS Vasculitis Associated with Waldenström Macroglobulinemia.

Waldenström macroglobulinemia (WM) is an indolent B cell lymphoproliferative disorder with monoclonal IgM secretion. We present a patient with WM who presented with multifocal acute cortical ischemic strokes and was found to have central nervous system (CNS) vasculitis. Workup was negative for cryoglobulins and hyperviscosity syndrome. Immunosuppression with intravenous steroids and cyclophosphamide stabilized the patient's mental status and neurologic deficits. On followup over 7 years, patient gained independence from walking aids and experienced no recurrences of CNS vasculitis. To our knowledge, CNS vasculitis in a WM patient, in the absence of cryoglobulins, has not been reported. Immunosuppression is the preferred treatment.

Calcified parenchymal central nervous system cysticercosis and clinical outcomes in epilepsy.

OBJECTIVE: This study aimed to compare clinical outcomes including seizure frequency and psychiatric symptoms between patients with epilepsy with neuroimaging evidence of past brain parenchymal neurocysticercosis infection, patients with other structural brain lesions, and patients without structural neuroimaging abnormalities. MATERIAL AND METHODS: The study included retrospective cross-sectional analysis of all patients treated for epilepsy in a community-based adult neurology clinic during a three-month period. RESULTS: A total of 160 patients were included in the analysis, including 63 with neuroimaging findings consistent with past parenchymal neurocysticercosis infection, 55 with structurally normal brain neuroimaging studies, and 42 with other structural brain lesions. No significant differences were detected between groups for either seizure freedom (46.03%, 50.91%, and 47.62%, respectively; p=0.944) or mean seizure frequency per month (mean=2.50, S.D.=8.1; mean=4.83, S.D.=17.64; mean=8.55, S.D.=27.31, respectively; p=0.267). Self-reported depressive symptoms were more prevalent in those with parenchymal neurocysticercosis than in the other groups (p=0.003). No significant differences were detected for prevalence of self-reported anxiety or psychotic symptoms. CONCLUSIONS: Calcified parenchymal neurocysticercosis results in refractory epilepsy about as often as other structural brain lesions. Depressive symptoms may be more common among those with epilepsy and calcified parenchymal neurocysticercosis; consequently, screening for depression may be indicated in this population.

Past Cigarette Smoking Is More Common among Those with Cholinergic Than Noncholinergic Dementias.

Background. Patients with progressive dementing disorders associated with cortical cholinergic dysfunction gradually develop cholinergic deficits many years before symptom onset and may begin to smoke cigarettes during midlife as a form of self-medication. The aim of this study was to compare self-reported past smoking rates between those with and without cholinergic dementias, to determine if those who developed cholinergic dementias were more likely to smoke during midlife than those who did not. Methods. Retrospective cross-sectional study of past smoking status among patients treated at an outpatient clinic during a three-year period. Results. A total of 440 patients were evaluated during the study period, including 224 with cholinergic dementias and 216 with noncholinergic dementias and controls. Past smoking rates were greater among those with cholinergic dementias compared to those without cholinergic dementias (43.92% versus 26.96%, P = 0.012). Additionally, smokers with cholinergic dementias reported significantly greater mean pack-years of smoking (P = 0.038). Conclusions. Greater midlife smoking rates and greater pack-years of smoking were associated with cholinergic dementias. These results suggest midlife smoking may be an early indicator for those developing brain cholinergic deficits related to progressive dementing disorders and support initiating treatment prior to symptom onset in cholinergic dementias.

Diffuse leukoencephalopathy and subacute parkinsonism as an early manifestation of systemic lupus erythematosus.

Parkinsonism in SLE is rare. Diffuse leukoencephalopathy is equally uncommon and is associated with a poor prognosis. We present a single case of a 50-year-old Filipino man who presented with a generalized discoid rash after starting lisinopril. The rash persisted despite discontinuation of lisinopril, and over the next three months, he developed rapidly progressive parkinsonism. Brain MRI showed symmetric confluent T2-hyperintensities involving the white matter and basal ganglia. Four of the 11 American College of Rheumatology criteria for the classification of SLE were met. A rheumatologist made a diagnosis of SLE with cutaneous and central nervous system involvement. Significant neurologic and radiologic improvement occurred following treatment with IV steroids followed by a prolonged taper. This report highlights a case of subacute parkinsonism with a diffuse leukoencephalopathy as an early manifestation of SLE which resulted in a good recovery following treatment with only immunosuppressive therapy.

Greater frequency of depression associated with chronic primary headaches than chronic post-traumatic headaches.

OBJECTIVE: To compare the prevalence of co-morbid depression between patients with chronic primary headache syndromes and chronic posttraumatic headaches. METHOD: A prospective cross-sectional analysis of all patients presenting sequentially to a community-based general neurology clinic during a 2-year period for evaluation of chronic headache pain was conducted. Headache diagnosis was determined according to the International Headache Society's Headache Classification criteria. Depression was determined through a combination of scores on the clinician administered Hamilton Rating Scale for Depression and patients' self-report. An additional group of patients who suffered traumatic brain injuries (TBI) but did not develop post-traumatic headaches was included for comparison. RESULTS: A total of 83 patients were included in the study: 45 with chronic primary headaches (24 with chronic migraine headaches, 21 with chronic tension headaches), 24 with chronic post-traumatic headaches, and 14 with TBI but no headaches. Depression occurred less frequently among those with chronic post-traumatic headaches (33.3%) compared to those with chronic migraine (66.7%) and chronic tension (52.4%) headaches (Chi-Square = 7.68; df = 3; p = 0.053), and did not significantly differ from TBI patients without headaches. A multivariate logistic regression model using depression as the outcome variable and including headache diagnosis, gender, ethnicity, and alcohol and illicit substance use was statistically significant (Chi-Square = 27.201; df = 10; p < 0.01) and identified primary headache (migraine and tension) diagnoses (Score = 7.349; df = 1; p = 0.04) and female gender (Score = 15.281; df = 1; p < 0.01) as significant predictor variables. The overall model accurately predicted presence of co-morbid depression in 74.7% of the cases. CONCLUSIONS: Co-morbid depression occurs less frequently among patients with chronic post-traumatic headaches and TBI without headaches than among those with chronic primary headaches.

Case of levodopa toxicity from ingestion of Mucuna gigantea.

Hawai'i is home to 1000 native species of flowering plants. Mucuna gigantea is one such Hawaiian species which has been studied as affordable sustenance and as a cover crop in developing countries. Mucuna gigantea and other Mucuna species (spp.) in general, are known to contain natural levodopa and its utility in the treatment of Parkinson's Disease has also been evaluated. Levodopa is converted in the periphery into dopamine which can then act on dopamine receptors to cause nausea, vomiting, arrhythmias, and hypotension. We describe a case in which a patient presents with abdominal pain, nausea, and vomiting after legume ingestion. The bean was ultimately identified as Mucuna gigantea and the patient was diagnosed with levodopa-induced gastrointestinal toxicity from consumption of the legume. A literature review was conducted using the database search engines, Biological Abstracts and PubMed, with a broad combination of keywords of which include "mucuna, "gigantean," "levodopa," "l-dopa," "toxicity," and the association between Mucuna gigantea ingestion and levodopa toxicity is discussed. These findings expand the differential diagnosis of abdominal pain associated with nausea and vomiting in the correct clinical context.

Coiled internal carotid arteries associated with bilateral sequential strokes.

The risk of stroke and management of coiling of the cervical internal carotid artery in the absence of an atherosclerotic carotid bulb lesion is unclear. We report a case of an otherwise healthy 39-year-old woman who developed bilateral sequential strokes associated with bilateral coiled internal carotid arteries. We discuss the risk of stroke and management of coiled carotid arteries as they relate to the patient presented.

Frontal-executive versus posterior-perceptual mental status deficits in early-onset dementias.

BACKGROUND: Compared to late-onset dementias, early-onset dementias (EODs) may have greater focal cognitive involvement with differences in frontal-executive compared to posterior-perceptual deficits. OBJECTIVE: This study evaluated whether mental status screening based on this frontal-posterior axis can distinguish EODs. METHODS: Twenty-three patients each with early-onset Alzheimer's disease (eAD), frontotemporal dementia (FTD), or subcortical ischemic vascular disease (SIVD), and 20 normal controls underwent the Frontal Assessment Battery (FAB) and the Perceptual Assessment Battery (PAB). RESULTS: Compared to controls, SIVD and FTD groups were impaired on the FAB whereas eAD and SIVD groups were impaired on the PAB. The FAB/PAB ratio further differentiated the groups (F(3,85) = 26.49, P < .001). For sensitivities and specificities of 93%, a cut-off score of 1.25 on the FAB/PAB distinguished eAD, and a cut-off of 0.83 distinguishing FTD. CONCLUSION: Although preliminary, this study indicates that mental status screening based on frontal versus posterior cortical functions may help clinicians diagnose EODs.

Content-specific delusions from right caudate lacunar stroke: association with prefrontal hypometabolism.

Patients with caudate lesions can present with content-specific delusions, possibly due to disruption of frontosubcortical circuits connecting the frontal lobes with the basal ganglia. This study included eight patients who presented with content-specific delusions after right caudate stroke and an equal number of matched controls, without stroke or delusions. Compared to controls, patients with caudate lesions performed less well on tests of memory, abstract reasoning, and frontal executive functions, and had significantly reduced metabolism in the inferior prefrontal cortex. These patients suggest an etiological relationship between alterations in inferior prefrontal functions and the development of content-specific delusions.

Positron emission tomography facilitates diagnosis of early-onset Alzheimer's disease.

BACKGROUND: Clinical positron emission tomography (PET) may help in the evaluation of presenile patients with memory complaints for the presence of Alzheimer's disease (AD). METHODS: Clinical PET scans from 27 patients with clinically probable AD and early ages of onset (<65 years) were compared to PET scans from 27 age-matched controls presenting with memory complaints, but without dementia or mild cognitive impairment. RESULTS: Compared to controls, the AD patients had significant frontal, temporal and parietal hypometabolism bilaterally, and AD diagnosis correlated with left temporal and right temporoparietal hypometabolism. The sensitivity of temporoparietal hypometabolism for AD was 92.6%, the specificity 85.2%. CONCLUSION: Clinical PET imaging helps distinguish early-onset AD from patients with memory complaints not meeting criteria for dementia or mild cognitive impairment.

Cognitive performance after lacunar stroke correlates with leukoaraiosis severity.

BACKGROUND: This study investigates the effect of leukoaraiosis on patients presenting with cognitive impairment after lacunar stroke. METHODS: Fourty-six patients with cognitive impairment and newly discovered lacunar stroke detected by brain magnetic resonance imaging underwent neuropsychological testing. RESULTS: Patients with both lacunar infarct and leukoaraiosis performed less well on cognitive measures, compared to those with lacunar infarcts alone. Additionally, leukoaraiosis severity inversely correlated with cognitive performance. CONCLUSIONS: In patients with lacunar stroke, presence of leukoaraiosis is associated with worse performance in multiple cognitive domains. These findings suggest lacunar infarcts plus leukoaraiosis is a common etiology for vascular dementia.

Cognitive differences between early- and late-onset Alzheimer's disease.

Although neuropathologic studies showed that early-onset Alzheimer's disease (EAD) and "senile dementia" were indistinguishable, clinical studies suggested that EAD and late-onset Alzheimer's disease (LAD) were cognitively distinct. We sought to investigate whether EAD and LAD are cognitively different by comparing patients at the extremes of the ages of onset in order to maximize features that might separate them. We compared 44 men with EAD (age of onset less than 65 years) with 44 men with LAD (age of onset 84 years or older) on an intake cognitive screening examination on initial presentation. The EAD and LAD groups did not differ on dementia or most cognitive variables. Compared with EAD, the LAD group had worse verbal fluency and motor-executive functions. These differences disappeared when age differences were taken into account. We conclude that Alzheimer's disease is a clinically heterogeneous disorder whose manifestations can vary with age of onset. These differences indicate age-related vulnerabilities in this disease.

Depressive and memory symptoms as presenting features of spinocerebellar ataxia.

The spinocerebellar ataxias (SCA) can be manifested in neuropsychiatric symptoms. Among 76 SCA patients presenting to a university ataxia center, depressive symptoms characterized SCA3. Memory symptoms occurred across all SCA groups with relative sparing of SCA6. These differences in neuropsychiatric symptoms suggest the subtype of SCA and the corresponding neuropathological involvement.