Primary Sjogren's syndrome with central nervous system involvement

To describe the clinical, laboratory, and radiological features of Primary Sjogren's syndrome [PSS] with central nervous system [CNS] involvement. A retrospective case series of 12 female patients with PSS and CNS involvement at King Faisal Specialist Hospital and Research Center, Riyadh, Kingdom of Saudi Arabia from 1991-2009. The diagnosis of PSS is defined by the American-European Diagnostic Criteria. We analyzed the clinical, radiological, and immunological features. The mean age was 40 years [range 16-58 years]; all patient were females and presented with active neurological symptoms. The neurological involvement preceded the classic sicca symptoms [33%]. Eight patients [66%] presented with myelopathy, 9 patients [75%] had optic neuritis, and the rest had variable neurological signs. Immunological tests [anti-Sjogren's syndrome A and anti-Sjogren's syndrome B] were high in 7 patients [58%]. Minor salivary gland biopsy revealed inflammatory cell infiltrate in 11 patients [92%]. Brain MRI showed scattered white matter changes in 7 patients [58%]. Spine MRI showed multiple foci of hyperintensity in T2-weighted image in 6 patients [50%], and long segment of hyperintensity at the cervical spinal cord in 2 patients [16%]. Our findings demonstrate that CNS involvements in PSS have great clinical variability and could precede the classic sicca symptoms by years. Primary Sjogren's syndrome can mimic multiple sclerosis [primary progressive multiple sclerosis or relapsing remitting multiple sclerosis], therefore a screening test for PSS should be considered in suspected cases. A well-defined management protocol awaits studies with larger case numbers

Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy in Arabs

To investigate the Notch 3 mutation spectrum in Arab patients with cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy CADASIL, which is an inherited cerebrovascular disease characterized by recurrent subcortical ischemic stroke starting in the third or fourth decade. Complete neurological evaluation and sequencing of the Notch 3 gene were carried out at King Faisal Specialist Hospital and Research Centre in 2007 on 2 families from Riyadh, Kingdom of Saudi Arabia and Sudan affected by CADASIL. The index cases had adult onset stroke, vascular dementia, behavioral and psychiatric symptoms and accelerated deaths. In both families, abnormal magnetic resonance imaging findings were detected in symptomatic and asymptomatic individuals. All Notch 3 exons were screened for mutations in both families and no known or novel mutation could be found; although, in one family the brain biopsy showed the typical granular osmiophilic material deposition and the vascular smooth muscle cells. This is the first 2 cases of CADASIL in Arabs, which occur without an obvious Notch 3 mutation

neurobiology of aging

This review describes briefly some of the age-associated changes in neural function. A decrease in the activity of neurotransmission system has been observed during normal aging. The aged brain is characterized by neuronal loss and atrophy, and the presence of amyloidal plaques and neurofibrillary tangles. Calcium dishomeotasis and changes in signal transduction, changes in growth factor response and changes in oxidative metabolism are altered with aging. The cholinergic system shows the most important alteration and this is possibly associated with age-related neurodegenerative disease. These changes suggest a strong correlation between normal and pathological aging