Seizures in systemic lupus erythematosus: A scoping review.

Systemic lupus erythematosus is a systemic autoimmune disease that affects the central nervous system, either by direct neuronal damage, injury to brain vessels, or by pathogenic mechanisms indirectly induced by immune mechanisms related to the production and deposition of immune complexes. The prevalence of explicit episodes of seizures among SLE patients, varies from 2 to 8%. In some cases, patients with positivity for antiphospholipid or anti-ß2 glycoprotein antibodies are found to be more prone to exhibit seizures compared to seronegative patients, other subjects at risk are carries of gene abnormalities codifying for ion channels. The exclusion of vasculitis or thrombosis is required for accurate treatment, imaging studies and alternative sequences are mandatory in patients with known SLE who present with a seizure. Several statements regarding SLE-related seizure remain to be decoded. In this scoping review we analyzed published information about prevalence, pathogenesis, clinical characteristics, diagnostic and therapeutic SLE patients that manifest a seizure, our objective is to provide with useful information for prompt diagnosis and individualized treatment.

Atypical forms of the osmotic demyelination syndrome.

Osmotic demyelination syndrome (ODS) is the damage over the central nervous system caused by several electrolytes, metabolic and toxic disorders. We aimed to describe cases of unusual forms of ODS. In a 9-year period, 25 consecutive patients with ODS (15 men; mean age 42 years) were registered in our referral institution, among them, four (16 %) with atypical neuroimaging findings were abstracted for this communication. None of them presented cardiorespiratory arrest, head trauma, seizures, neuromyelitis optica spectrum or contact with toxic chemicals. Case 1 was a 33-year-old alcoholic man without hypertension or electrolyte imbalance, who presented a classic central pontine myelinolysis (CPM) and a hemorrhage within the pons. Case 2 was a 34-year-old alcoholic man with hypoglycemia and hyponatremia who presented CPM and diffuse bihemispheric extrapontine myelinolysis (EPM) after correction of serum sodium. Case 3 was a 52-year-old woman with mild hypokalemia and hyponatremia (inadequately corrected), who presented a peduncular and cerebellar EPM. Case 4 was a 67-year-old woman who had a suicidal attempt with antidepressants and carbamazepine without impaired consciousness, who complicated with mild hyponatremia associated with a classical CPM and a spinal cord EPM. Case 2 died and the rest remained with variable neurological impairments at last follow-up visit. With modern neuroimaging, the so-called atypical forms of ODS may not be as rare as previously thought; however, they could have a more adverse outcome than the classical ODS.

Regulation of neural stem cell in the human SVZ by trophic and morphogenic factors.

The subventricular zone (SVZ), lining the lateral ventricular system, is the largest germinal region in mammals. In there, neural stem cells express markers related to astoglial lineage that give rise to new neurons and oligodendrocytes in vivo. In the adult human brain, in vitro evidence has also shown that astrocytic cells isolated from the SVZ can generate new neurons and oligodendrocytes. These proliferative cells are strongly controlled by a number of signals and molecules that modulate, activate or repress the cell division, renewal, proliferation and fate of neural stem cells. In this review, we summarize the cellular composition of the adult human SVZ (hSVZ) and discuss the increasing evidence showing that some trophic modulators strongly control the function of neural stem cells in the SVZ.

Choreoacanthocytosis in a Mexican family.

BACKGROUND: Choreoacanthocytosis (CHAC) (Online Mendelian Inheritance in Man accession No. 200150) is a hereditary neurodegenerative syndrome characterized by movement disorders, cognitive decline, myopathy, behavioral changes, and acanthocytosis and is caused by mutations in the VPS13A gene. OBJECTIVE: To describe the cases of 2 Mexican women with clinical and molecular characteristics compatible with CHAC. DESIGN: Case reports. Patients Choreoacanthocytosis was identified in 2 Mexican mestizo sisters with healthy consanguineous parents. Clinical manifestations began at different ages. RESULTS: The onset of signs and symptoms of CHAC in the proband was at age 32 years and was characterized by balancing problems followed by chorea, compulsive lip and tongue biting with buccolingual self-mutilation, dysarthria, dysphagia, and weight loss. The first clinical manifestations in the proband's sister occurred at age 45 years and included multiple motor and verbal tics, with coprolalia, followed by lip and tongue biting, self-mutilation, and chorea. The clinical findings in both sisters were remarkable for acanthocytosis that developed late, when neurologic changes were already evident. Mutation screening of the VPS13A gene revealed homozygosity for the frameshift mutation c.3556_3557dupAC in exon 33. Currently, the proband's sister, in whom neurologic defects developed 13 years after onset of CHAC in the proband, is the least affected. CONCLUSIONS: The same mutation of the VPS13A gene can be expressed differently in the same family. This observation confirms the notion that there is considerable heterogeneity in the clinical manifestation of CHAC.

Hypertensive intracerebral hemorrhage in the very elderly.

BACKGROUND: The number of persons reaching the age 80 years and over is increasing in most populations. Literature focusing on hypertensive intracerebral hemorrhage (ICH) in this age group is lacking. Therefore, we aimed to analyze the main clinical characteristics of ICH of the advanced old age, in the context of hypertension. METHODS: From 1999 to 2003 we studied 56 hypertensive very elderly patients presenting with ICH (50% women; age 80-99 years). As controls, 168 hypertensive gender-matched persons with ICH, aged <80 years, were randomly selected by a 3:1 factor for clinical comparisons. RESULTS: Compared with their younger counterparts, the very elderly patients had a trend for fewer cases of obesity (34 vs. 49%, p = 0.05) and diabetes mellitus (12 vs. 24%, p = 0.06), had lower systolic, diastolic and mean blood pressure measures (in all, p < 0.01) and more cases with hematoma extension into ventricles (p = 0.02). Thalamic hemorrhage was more frequent in the very elderly patients than in controls (43 vs. 28%, p = 0.04). In multivariate analysis, age, Glasgow coma scale score at hospital admission, ICH volume and infratentorial location were independent predictors of in-hospital mortality, in all persons combined. In the very elderly group exclusively, Glasgow coma scale score was the only factor independently associated with mortality. CONCLUSIONS: ICH occurring in hypertensive patients aged > or =80 years has several differences from that seen in younger people; however, these differences do not seem to impact on early outcome.