No cerebral or cervical venous insufficiency in US veterans with multiple sclerosis.

OBJECTIVE: To determine if chronic cerebral venous insufficiency exists in patients with multiple sclerosis (MS) using ultrasonography and 4-dimensional color Doppler ultrasonography examination and unverified criteria proposed by Zamboni et al. DESIGN: Patients with MS and clinically isolated syndrome were matched by age and sex with subjects with migraine or no neurological disease. All subjects underwent gray-scale, color, and spectral Doppler ultrasonography examination of the internal jugular veins (IJVs), vertebral veins, and deep cerebral veins for stenosis, absence of signal, and reflux. SETTING: Academic MS center. PATIENTS: All patients with MS fulfilled revised McDonald criteria for the diagnosis of MS. Patients with clinically isolated syndrome exhibited a typical transient focal neurological deficit and had magnetic resonance imaging lesions typical of MS. Control subjects were recruited from the VA migraine clinic or staff. MAIN OUTCOME MEASURES: Five parameters of venous outflow used by Zamboni et al were examined: (1) IJV or vertebral vein reflux, (2) deep cerebral vein reflux, (3) IJV stenosis, (4) absence of flow in IJVs or vertebral veins, and (5) change in cross-sectional area of the IJV with postural change. RESULTS: There was no significant difference in the number and type of venous outflow abnormalities in patients with MS compared with controls. CONCLUSION: This study does not support the theory that chronic cerebral venous insufficiency exists in MS.

Multiple sclerosis and chronic cerebrospinal venous insufficiency: a critical review.

Chronic cerebrospinal venous insufficiency (CCSVI) was recently proposed as a contributing factor in the pathology of multiple sclerosis. This concept has gained remarkable attention, partly because endovascular neurointervention has been suggested as a treatment strategy. This review summarizes available evidence and provides a critical analysis of the published data. Currently, there is inconclusive evidence to support CCSVI as an etiological factor in patients with multiple sclerosis. Endovascular procedures should not be undertaken outside of controlled clinical trials.

A middle-aged woman with logopenic progressive aphasia as a precursor of Alzheimer's disease: case report and review of the literature.

PRIMARY PROGRESSIVE APHASIA IS A NEURODEGENERATIVE DISORDER THAT WAS RECENTLY CLASSIFIED INTO THREE TYPES: fluent (semantic), nonfluent, and logopenic. The logopenic variant is the least common one and is closely related to Alzheimer's disease in comparison to the other two variants that are closely related to frontotemporal dementia. We report the case of a middle-aged woman who presented to our center with progressive aphasia that was undiagnosed for two years. The patient's neurological evaluation including positron emission tomography is consistent with a logopenic variant of primary progressive aphasia.

Isolated central nervous system vasculitis associated with antiribonuclear protein antibody.

We describe the case of a young woman who was referred to a tertiary care center with unexplained subacute progressive encephalopathy preceded by long-standing severe headaches. Her extensive workup was remarkable for abnormal intracranial angiography suggestive of small- and medium-vessel vasculitis, persistently elevated protein in the cerebrospinal fluid and persistently high titers of antiribonuclear protein antibody. The patient showed a modest response to intravenous high-dose steroids. We propose that the patient's neurologic disease is secondary to immune-mediated central nervous system vasculitis, possibly as an initial manifestation of mixed connective tissue disease.

Hypnopompic seizures.

The relationship between epilepsy and sleep is complex and bidirectional. Ictal awakening is probably a common and well-described phenomenon. In this small observational study we describe arousal from sleep as the only, or at least main, manifestation of some epileptic seizures. We coin the term "hypnopompic seizures" to describe this entity. Five patients with intractable epilepsy were monitored by continuous video-electroencephalogram. Four of them had left temporal lobe epilepsy and one patient had generalised epilepsy. Hypnopompic seizures accounted for 30-100% of their seizure types captured during monitoring. All the seizures occurred during stage II sleep and were brief. Hypnopompic seizures are extremely subtle and may be underdiagnosed and underreported. Future larger studies are needed to shed some light on this unique entity and its neuropathophysiology. Epileptologists should be aware of this type of seizure and careful review of electroencephalograms during the transition from sleep to arousal is imperative to capture these seizures. Physicians, patients and families also need to be aware of such a subtle manifestation of seizures. Improved awareness of hypnopompic seizures and subtle seizures, in general, help guide accurate and early diagnosis, thorough monitoring and appropriate management.

Immunopathogenesis of multiple sclerosis: new insights and therapeutic implications.

Multiple sclerosis (MS) is an inflammatory, demyelinating, and neurodegenerative disorder of the CNS. The etiology of MS remains unknown. However, it is well established that immune dysregulation plays a critical role in the neuropathogenesis of this disorder. In this review, we discuss the current hypotheses concerning the complex cellular and molecular interactions involved in the immunopathogenesis of MS. Although CD4 T lymphocytes have long been considered the critical cellular factor in the immunopathology of MS, the role of other cell types has also recently been investigated. It appears that the spatial distribution of CD4 and CD8 cells in MS lesions is distinct. Yet another T-lymphocyte subset, γ/δ T cells, can be detected in very early MS lesions. The prevalent dogma suggests that CD4 helper T (TH) type 1 cells release cytokines and inflammatory mediators that cause tissue damage, while CD4 TH2 cells might be involved in modulation of these effects. However, a mounting body of evidence suggests that additional T-cell subsets, including TH17 cells, CD8 effector T cells, and CD4 CD25 regulatory T cells, also affect disease activity. In addition, clinical and paraclinical data are accumulating on the prominent role of B lymphocytes and other antigen-presenting cells in MS neuropathogenesis. Given these observations, new therapeutic interventions for MS will need to focus on resetting multiple components of the immune system.

Optic neuritis presenting with amaurosis fugax.

Optic neuritis usually presents with rapid and gradual loss of vision that is either complete or incomplete, and typically associated with retro-orbital pain. To our knowledge there have been no documented reports of optic neuritis presenting with multiple episodes of amaurosis fugax, the sudden and transient loss of vision lasting seconds to minutes. We report here the case of a young woman with a possible diagnosis of demyelinating left optic neuritis that presented solely with multiple episodes of brief and transient sudden loss of vision. Ophthalmological exams were normal between episodes of vision loss. The patient's magnetic resonance imaging of the brain showed a subtle enhancement of the left optic nerve along with multiple periventricular lesions, highly suggestive of a demyelinating disease. The frequent episodes of visual loss resolved completely with high dose parenteral steroids. Neurologists and other clinicians should be aware of this unusual presentation of optic neuritis as treatment modalities differ greatly from other causes of amaurosis fugax.