Intraventricular neurocysticercosis causing obstructing hydrocephalus.

Neurocysticercosis is a parasitic infection caused by the larval stage of the pork tapeworm Taenia solium. Diagnostic criteria for neurocysticercosis are based on clinical manifestations, neuroimaging findings, and epidemiologic exposure. Treatment is tailored to acute symptoms. If increased intracranial pressure is present, then surgical intervention and/or corticosteroids may be used. If seizures are present, then antiepileptic drugs are used for therapy. Antiparasitic therapy is warranted for patients with viable and/or degenerating cysts in the brain parenchyma on neuroimaging. We present a case report of neurocysticercosis causing obstructive hydrocephalus requiring a ventriculoperitoneal shunt.

Urinary urge seizure semiology localization by intracranial monitoring.

Seizures may present with many different symptom complexes. Seizure semiologies have localization value when contemplating surgical therapies. Epilepsy presenting as a desire to micturate is exceedingly rare, with only a handful of published cases. We present a patient who had initially unexplained childhood urinary urge that progressed into lapses of memory and confusion that was eventually diagnosed as epilepsy in adulthood. Due to refractoriness to medical management, our patient's epileptic focus was localized through both noninvasive and invasive subdural electrode arrays to the nondominant medial temporal lobe and successfully treated with an amygdalohippocampectomy. Only one other case of ictal urinary urgency seizures has been recorded with intracranial electroencephalogram monitoring in the literature.

Dural arteriovenous fistula as a treatable dementia.

Dementia is a chronic loss of neurocognitive function that is progressive and irreversible. Although rare, dural arteriovenous fistulas (DAVFs) could present with a rapid decline in neurocognitive function with or without Parkinson-like symptoms. DAVFs represent a potentially treatable and reversible cause of dementia. Here, we report the case of an elderly woman diagnosed with a DAVF after presenting with new-onset seizures, deteriorating neurocognitive function, and Parkinson-like symptoms.

Right hemispheric reversible cerebral vasoconstriction syndrome in a patient with left hemispheric partial seizures.

We report a right-handed 19-year-old girl who developed reversible cerebral vasoconstriction syndrome (RCVS) lateralized to the right hemisphere with simultaneous new-onset left hemispheric seizures. RCVS, typically more diffuse, was lateralized to one of the cerebral hemispheres.

Seronegative neuromyelitis optica after cardiac transplantation.

We report a case of a 42-year-old man who presented with progressive weakness and blindness over the course of several months and met criteria for seronegative neuromyelitis optica. This presentation was in the setting of immunosuppression following cardiac transplant. No infectious causes were found within the neuroaxis, and he ultimately died with complete blindness, quadriplegia, and respiratory failure attributed to panmyelitis and brain stem inflammation despite aggressive therapies.

Paraneoplastic cerebellar ataxia and the paraneoplastic syndromes.

Paraneoplastic cerebellar ataxia, also known as paraneoplastic cerebellar degeneration, is one of the wide array of paraneoplastic neurological syndromes in which neurological symptoms are indirectly caused by an underlying malignancy, most commonly gynecological, breast, or lung cancer or Hodgkin's lymphoma. We describe a patient with severe cerebellar dysfunction attributed to a paraneoplastic neurological syndrome. The case highlights the need to look for paraneoplastic syndromes-both to discover malignancies early, at a treatable stage, and, as in our case, to address very distressing symptoms for the patient's relief even if the malignancy is not curable.

Ascending paralysis associated with HIV infection.

We present two patients with a high viral load of HIV-1 who developed symptoms of ascending paralysis leading to respiratory failure and autonomic instability. One patient had symptom improvement with highly active antiretroviral therapy (HAART) and a subsequent decrease in viral load. The other patient improved with intravenous immunoglobulin therapy and did not show much improvement on HAART alone. There are several proposed mechanisms for peripheral neuropathies seen in HIV-infected patients, including a direct action of HIV on the nerve by neurotropic strains or formation of autoantibodies against nerve elements. The comparison of the response to different therapies in these two cases highlights the importance of understanding different pathophysiologies, as the treatment modality may differ.

Basilar artery thrombosis in the setting of antiphospholipid syndrome.

Antiphospholipid syndrome is an autoimmune disorder characterized by arterial or venous thrombosis, recurrent first-trimester pregnancy loss, and multiple additional clinical manifestations. We describe a man with severe atherosclerotic basilar artery stenosis and superimposed in situ thrombus who was found to have antiphospholipid syndrome.

Opsoclonus myoclonus syndrome: an unusual presentation for West Nile virus encephalitis.

A record number of West Nile virus (WNV) cases and fatalities seen in 2012 have brought to light the numerous manifestations of neuroinvasive disease. We report a case of opsoclonus myoclonus syndrome attributed to WNV and its clinical course after treatment with a combination of steroids and intravenous immunoglobulin. Our objective is to highlight opsoclonus myoclonus syndrome as a potential manifestation of WNV encephalitis.

Herpes simplex virus meningitis complicated by ascending paralysis.

A case of herpes simplex virus (HSV) meningitis complicated by ascending paralysis with almost complete recovery following antiviral treatment is reported. We present this case to illustrate the importance of including HSV-induced neuropathy in the differential diagnosis of acute neurologic symptoms following the viral illness.

Bilateral hippocampal atrophy in temporal lobe epilepsy: effect of depressive symptoms and febrile seizures.

PURPOSE: Neuroimaging studies suggest a history of febrile seizures, and depression, are associated with hippocampal volume reductions in patients with temporal lobe epilepsy (TLE). METHODS: We used radial atrophy mapping (RAM), a three-dimensional (3D) surface modeling tool, to measure hippocampal atrophy in 40 patients with unilateral TLE, with or without a history of febrile seizures and symptoms of depression. Multiple linear regression was used to single out the effects of covariates on local atrophy. KEY FINDINGS: Subjects with a history of febrile seizures (n =15) had atrophy in regions corresponding to the CA1 and CA3 subfields of the hippocampus contralateral to seizure focus (CHC) compared to those without a history of febrile seizures (n = 25). Subjects with Beck Depression Inventory II (BDI-II) score ≥ 14 (n = 11) had atrophy in the superoanterior portion of the CHC compared to subjects with BDI-II <14 (n = 29). SIGNIFICANCE: Contralateral hippocampal atrophy in TLE may be related to febrile seizures or depression.

Temporal lobectomy: resection volume, neuropsychological effects, and seizure outcome.

OBJECTIVE: The goal of the work described here was to evaluate relationships among resection volume, seizure outcome, and cognitive morbidity after temporal lobectomy for intractable epilepsy. METHODS: Thirty patients with mesial temporal sclerosis were evaluated pre- and postoperatively with the Wechsler Adult Intelligence Scale III, Wechsler Memory Scale III, and three-dimensional coronal spoiled gradient recall acquisition MRI. Preoperative whole-brain volumes were calculated with Statistical Parametric Mapping. Resection volume was calculated by manual tracing. Systat was used for statistical analysis. RESULTS: All resections included the temporal tip, at least 1cm of the superior temporal gyrus, and 3 to 5cm of the middle and inferior temporal gyri. Left were significantly smaller than right temporal resections. Seizure-free patients had significantly larger resections. Immediate verbal memory was significantly worse after left temporal lobectomy. Surgical outcome and resection volume did not affect postoperative neuropsychological results. CONCLUSIONS: Dominant temporal lobe resections are associated with immediate verbal memory deficits. Larger resection volume was associated with improved seizure control but not worse cognitive outcome.

18F-FCWAY and 18F-FDG PET in MRI-negative temporal lobe epilepsy.

BACKGROUND: Positron emission tomography (PET) with (18)F-fluorodeoxyglucose (FDG) shows widespread hypometabolism even in temporal lobe epilepsy (TLE) patients with mesial temporal foci. (18)F-trans-4-fluoro-N-2-[4-(2-methoxyphenyl) piperazin-1-yl]ethyl-N-(2-pyridyl)cyclohexane carboxamide ((18)F-FCWAY) PET may show more specific 5-HT(1A)-receptor binding reduction in seizure initiation than in propagation regions. (18)FCWAY PET might be valuable for detecting epileptic foci, and distinguishing mesial from lateral temporal foci in MRI-negative patients with TLE. METHODS: We performed (18)F-FCWAY-PET and (18)F-FDG-PET in 12 MRI-negative TLE patients who had had either surgery or subdural electrode recording, and 15 healthy volunteers. After partial volume correction for brain atrophy, free fraction-corrected volume of distribution (V/f1) measurement and asymmetry indices (AIs) were computed. We compared (18)F-FCWAY-PET and (18)F-FDG-PET results with scalp video electroencephalography (EEG), invasive EEG, and surgical outcome. RESULTS: Mean (18)F-FCWAY V/f1, compared with normal controls, was decreased significantly in fusiform gyrus, hippocampus, and parahippocampus ipsilateral to epileptic foci, and AIs were significantly greater in hippocampus, parahippocampus, fusiform gyrus, amygdala, and inferior temporal regions. Eleven patients had clearly lateralized epileptogenic zones. Nine had congruent, and two nonlateralized, (18)F-FCWAY PET. One patient with bitemporal seizure onset had nonlateralized (18)F-FCWAY-PET. (18)F-FDG-PET showed congruent hypometabolism in 7 of 11 EEG-lateralized patients, bilateral hypometabolic regions in one, contralateral hypometabolism in one, as well as lateralized hypometabolism in the patient with bitemporal subdural seizure onset. Patients with mesial temporal foci tended to have lower superior and midtemporal (18)F-FCWAY V/f1 binding AI than those with lateral or diffuse foci. CONCLUSION: (18)F-FCWAY-PET can detect reduced binding in patients with normal MRI, and may be more accurate than (18)F-FDG-PET.

Temporal lobe epilepsy, depression, and hippocampal volume.

OBJECTIVE: To evaluate the relationship between hippocampal volume loss, depression, and epilepsy. BACKGROUND: There is a significantly increased incidence of depression and suicide in patients with epilepsy. Both epilepsy and depression are associated with reduced hippocampal volumes, but it is uncertain whether patients with both conditions have greater atrophy than those with epilepsy alone. Previous studies used depression measures strongly weighted to current state, and did not necessarily assess the influence of chronic major depressive disorder ("trait"), which could have a greater impact on hippocampal volume. METHODS: Fifty-five epilepsy patients with complex partial seizures (CPS) confirmed by electroencephalography (EEG) had three-dimensional (3D)-spoiled gradient recall (SPGR) acquisition magnetic resonance imaging (MRI) scans for hippocampal volumetric analysis. Depression screening was performed with the Beck Depression Inventory (BDI, 51 patients) and with the structured clinical inventory for DSM-IV (SCID, 34 patients). For the BDI, a score above 10 was considered mild to moderate, above 20 moderate to severe, and above 30 severe depression. MRI and clinical analysis were performed blinded to other data. Statistical analysis was performed with Systat using Student's t test and analysis of variance (ANOVA). RESULTS: There was a significant interaction between depression detected on SCID, side of focus, and left hippocampal volume. Patients with a diagnosis of depression and a right temporal seizure focus had significantly lower left hippocampal volume. A similar trend for an effect of depression on right hippocampal volume in patients with a right temporal focus did not reach statistical significance. CONCLUSIONS: Our results suggest that patients with right temporal lobe epilepsy and depression have hippocampal atrophy that cannot be explained by epilepsy alone.

Polysomnographic abnormalities in succinic semialdehyde dehydrogenase (SSADH) deficiency.

OBJECTIVES: Patients with SSADH deficiency, a disorder of chronically elevated endogenous GABA and GHB, were studied for sleep symptoms and polysomnography. We hypothesized that patients would have excessive daytime somnolence and decreased REM sleep. DESIGN: Polysomnography and MSLT were performed on patients enrolled for comprehensive clinical studies of SSADH deficiency. SETTING: Sleep studies were obtained in the sleep laboratories at CNMC and NIH. PATIENTS: Sleep recordings were obtained in 10 patients with confirmed SSADH deficiency. INTERVENTIONS: Thirteen overnight polysomnograms were obtained in 10 patients (7 male, 3 female, ages 11-27 y). Eleven MSLT studies were completed in 8 patients. MEASUREMENTS AND RESULTS: Polysomnograms showed prolongation of REM stage latency (mean 272 +/- 89 min) and decreased percent stage REM (mean 8.9%, range 0.3% to 13.8%). Decreased mean sleep latency was present in 6 of 11 MSLTs. CONCLUSIONS: SSADH deficiency is associated with prolonged latency to stage REM and decreased percent stage REM. This disorder represents a model of chronic GABA and GHB accumulation associated with suppression of REM sleep.

Usefulness of pulsed arterial spin labeling MR imaging in mesial temporal lobe epilepsy.

PURPOSE: Arterial spin labeling (ASL) is a developing magnetic resonance imaging (MRI) method for noninvasive measurement of cerebral blood flow (CBF). The purpose of this study was to evaluate the usefulness of ASL for detecting interictal temporal hypoperfusion in temporal lobe epilepsy (TLE). ASL-derived CBF measurements were compared with those derived from H(2)(15)O positron emission tomography (PET). METHODS: 11 normal controls and 10 patients with medically intractable TLE were studied. Pulsed ASL (PASL) with quantitative imaging of perfusion using a single subtraction, second version (QUIPSS II) was performed in all subjects and H(2)(15)O PET was performed in patients. Regional CBF values in the mesial and lateral temporal lobes were measured utilizing quantitative analysis of perfusion images. A perfusion asymmetry index (AI) was calculated for each region. RESULTS: In patients, mean CBF in the mesial temporal lobe was not significantly different between PASL and H(2)(15)O PET, and ipsilateral mesial temporal CBF was lower than contralateral CBF with both techniques. PASL detected significant mesial temporal perfusion asymmetry agreeing with EEG laterality in four patients. H(2)(15)O PET found ipsilateral interictal hypoperfusion in three. Both scans found unilateral hypoperfusion in one patient with bilateral EEG discharges. CONCLUSIONS: Pulsed ASL may be a promising approach to detecting interictal hypoperfusion in TLE. This method has potential as a clinical alternative to H(2)(15)O PET due to noninvasiveness and easy accessibility.

Constipation heralding neuroborreliosis: an atypical tale of 2 patients.

This is a report of 2 patients with Lyme disease who initially presented with severe constipation, which progressed to ascending muscular weakness resembling acute idiopathic polyneuritis, with neuropsychiatric symptoms, severe urinary retention, and hyponatremia. These symptoms resolved following proper antibiotic therapy.