Complex regional pain syndrome: A case report and review of the literature.

Background: Complex regional pain syndrome (CRPS) is a rare neuropathic pain disorder associated with severe pain, muscle weakness, limb edema and hyperhidrosis. Predisposing factors include fracture, surgery, stroke and spinal cord injury. CRPS may recur in the same limb or spread to other limbs to complicate management. Case Report: A 20-year old female with CRPS Type-I had sequential spread to all four limbs despite different treatment modalities, including medical therapy, nerve block, radiofrequency ablation and surgical sympathectomy. We discuss the therapeutic challenges and reviewed recent literature on current treatment options for CRPS Type-I. Conclusion: A multidisciplinary approach is needed for effective management of CRPS, and refractory disease may respond to intrathecal baclofen with morphine.

RésuméContexte: Le syndrome douloureux régional complexe (SDRC) est un trouble neuropathique rare associé à une douleur intense, une faiblesse musculaire, un dème des membres et une hyperhidrose. Les facteurs prédisposants comprennent la fracture, la chirurgie, l'AVC et les lésions de la moelle épinière. Le SDRC peut se reproduire dans le même membre ou se propager à d'autres membres pour compliquer la gestion. Rapport de cas: une femme de 20 ans atteinte du SDRC de type I s'est propagée séquentiellement aux quatre membres malgré différentes modalités de traitement, y compris une thérapie médicale, un bloc nerveux, une ablation par radiofréquence et une sympathectomie chirurgicale. Nous discutons des défis thérapeutiques et avons passé en revue la littérature récente sur les options de traitement actuelles pour le SDRC de type I. Conclusion: Une approche multidisciplinaire est nécessaire pour une gestion efficace du SDRC, et la maladie réfractaire peut répondre au baclofène intrathécal avec de la morphine.

The sympathetic skin response in diabetic neuropathy and its relationship to autonomic symptoms.

OBJECTIVE: To examine the utility of the sympathetic skin response SSR as a measure of impaired autonomic function among diabetic patients in Saudi Arabia. METHODS: In this case-control study, baseline SSR was obtained from 18 healthy subjects, followed by nerve conduction studies, and SSR testing on a consecutive cohort of 50 diabetic patients with peripheral neuropathy. The SSR in diabetic patients was compared between those with autonomic neuropathy and those without autonomic neuropathy. This study was conducted at the King Khaled University Hospital, Riyadh, Saudi Arabia, from June 2006 to June 2007. RESULTS: The SSR was present in all healthy subjects, and in 32 diabetic patients. Among 16 patients with autonomic neuropathy, the SSR was absent in 14 and present in 2, while 4 of 34 patients lacking evidence of autonomic neuropathy had absent SSR. Using Fisher's exact test, we found a strong association between absent SSR and autonomic neuropathy p<0.001, however, not with age or duration of diabetes mellitus. As a diagnostic test of autonomic neuropathy, the SSR had a sensitivity of 87.5%, a specificity of 88.2%, a positive predictive value of 77.8%, and a negative predictive value of 93.7%. CONCLUSION: Absence of the SSR is a reliable indicator of autonomic neuropathy among patients with diabetes mellitus in Saudi Arabia.

Myasthenia gravis: presentation and outcome in 104 patients managed in a single institution.

BACKGROUND AND OBJECTIVES: Few studies have attempted to delineate the clinical profile of myasthenia gravis (MG) among people of Arab ancestry. Therefore, we sought to clarify the clinical profile, the outcome of treatment and the role of thymectomy in non-thymomatous MG in Saudi Arabia. PATIENTS AND METHODS: We retrospectively studied 104 patients followed over a mean period of 7.2 years (range, 1 to 22 years) at the King Khaled University Hospital, Riyadh, Saudi Arabia. Disease outcomes were compared among thymectomized and non-thymectomized patients according to the post-intervention status criteria of the Myasthenia Gravis Foundation of America (MGFA). RESULTS: Age of onset was 22.5+/-9.3 years (meanA+/-SD) in females and 28.2+/-15.9 years in males, with peaks in the second and third decades among females and the third and fourth decades among males. At diagnosis, a majority of patients had moderate generalized weakness, equivalent to MGFA class III severity. After medical treatment with or without thymectomy, 9.6% of all patients had achieved complete stable remission, 3.8% had pharmacological remission, 27.9% had minimal manifestations, 23.1% were improved, 20.2% were unchanged and 15.4% were worse. Only thymectomized patients without a thymoma achieved remission, a significant benefit over those who had no thymectomy (P=.02). CONCLUSION: MG presents at a younger age among Saudi Arabs compared to other racial groups. Thymectomy conferred significant benefits towards achievement of remission.

Genetics of ischemic stroke.

Stroke is the third leading cause of death and a major cause of disability worldwide. Most cases of ischemic stroke are attributable to hypertension and other risk factors, but in over 20% of cases, the cause is unknown. Recent research has implicated some novel genes in the etiology of ischemic stroke, including genes for soluble epoxide hydrolase (sHE), 5-lipoxygenase activating protein (FLAP) and phosphodiesterase 4D (PDE4D). Moreover, thrombophilic states such as prothrombin G20210A mutation and factor V Leiden are now known to cause arterial stroke as well as venous thrombosis. Meanwhile, the recent availability of enzyme replacement therapy for Fabry disease and the proven benefits of regular blood transfusion in certain patients with sickle cell disease have greatly altered the outlook of these devastating inherited disorders. Thus, our understanding of the role of genetic factors in stroke raises the prospects for accurate assessment of future stroke risk among susceptible individuals, in whom early preventive measures may be life-saving. Further research into the genetics of stroke will clearly compliment ongoing national and international efforts to reduce the global burden of stroke.

Multiple sclerosis with recurrent meningitis.

Multiple sclerosis is an autoimmune demyelinating disease that is rarely associated with aseptic meningitis. However, certain syndromes causing aseptic meningitis are often associated with central nervous system demyelination that mimics multiple sclerosis (MS). Since many of these syndromes are potentially treatable, unmasking an alternative diagnosis is essential whenever an MS-like illness and recurrent meningitis are encountered in the same patient. Yet, the search for an alternative diagnosis may be elusive sometimes, despite extensive and appropriate investigations. We present a young woman with an MS-like illness associated with recurrent meningitis over a 7-year period. After an exhaustive evaluation, we conclude that recurrent meningitis is an atypical manifestation of MS. If neurologists would appreciate this point, unrewarding and costly investigations may be avoided and appropriate therapy instituted when similar cases are encountered in clinical practice.

Activation of microglia in mice spinal cords following passive transfer of purified IgG from patients with amyotrophic lateral sclerosis.

OBJECTIVE: To determine whether IgG from amyotrophic lateral sclerosis (ALS) patients could cause activation of microglia, proliferation of astrocytes, and infiltration by lymphocytes within mice spinal cords. METHODS: A group of 5 mice received injections of IgG purified from sera of patients with ALS. A control group of 5 mice received IgG from healthy humans, whilst a third group of 2 mice served as non-injected controls. One mouse served as a positive control and was injected with lipopolysaccharide, a known activator of microglia. Mice were culled after one week, for immunocytochemistry of spinal cord sections to localize the complement receptor CD11b on activated microglia, glial fibrillary acidic protein on astrocytes, and CD4 and CD8 receptors on lymphocytes. Histological examination was used to determine the presence of inflammatory reaction. This work was conducted at the Institute of Neurology, Queen Square London, United Kingdom, from January to July 2004. RESULTS: There was no significant difference in activation of microglia between mice injected with ALS IgG and mice injected with control IgG (p = 0.631), although mice injected with ALS IgG exhibited greater microglial activation than non-injected mice (p = 0.044). Proliferation of astrocytes was not significantly different between the 3 groups. CD4 and CD8 lymphocytes were both absent in mice injected with ALS IgG, mice injected with control IgG and non-injected mice. CONCLUSION: Activation of microglia following passive transfer of IgG from ALS patients to mice represents a non-specific inflammatory response, rather than a primary mechanism for motor neuron degeneration.

Prestroke treatment of stroke risk factors: A cross-sectional survey in central Nigeria.

BACKGROUND: Stroke can be prevented with treatments targeted at hypertension, diabetes mellitus, hyperlipidemia and atrial fibrillation, but this is often hampered by under-diagnosis and under-treatment of those risk factors. The magnitude of this problem is not well-studied in sub-Saharan Africa. MATERIALS AND METHODS: We conducted a cross-sectional survey of stroke patients at a tertiary hospital during January 2010 to July 2013 to determine patient awareness of a pre-existing stroke risk factor and prior use of anti-hypertensive, anti-diabetic, antiplatelet and lipid-lowering agents. We also investigated whether gender and school education influenced patient awareness and treatment of a stroke risk factor prior to stroke. RESULTS: Three hundred and sixty nine stroke patients presented during the study period, of which 344 eligible subjects were studied. Mean age at presentation (±SD) was 55.8 ± 13.7 years, and was not different for men and women. Hypertension, hyperlipidemia, diabetes and atrial fibrillation were prevalent among 83.7%, 26.5%, 25.6% and 9.6% patients respectively. Awareness was high for pre-existing diabetes (81.8%) and hypertension (76.7%), but not for hyperlipidemia (26.4%) and atrial fibrillation (15.2%). Men were better educated than women (p = 0.002), and had better awareness for hyperlipidemia (37.3% versus 13.5%; p = 0.009). Men were also more likely to take drug treatments for a stroke risk factor, but the differences were significant. CONCLUSIONS: A high rate of under-diagnosis and under-treatment of hypertension, hyperlipidemia and atrial fibrillation contributes to the stroke burden in sub-Saharan Africa, especially among women. Public health measures including mass media campaigns could help reduce the burden of stroke.

NeuroAIDS in sub-Saharan Africa: a clinical review.

NeuroAIDS affects half of the 22 million people currently living with HIV/AIDS in sub-Saharan Africa, where cryptococcal meningitis alone is responsible for 504,000 deaths annually. A good understanding of NeuroAIDS may help improve disease-free survival in patients at risk and optimize resource utilization by caregivers. In this review, we aimed to provide a summary of major NeuroAIDS syndromes of relevance in Africa. We searched Medline for English language literature to identify relevant publications, using the search terms "NeuroAIDS" and "HIV AND nervous system." The most common NeuroAIDS syndrome is HIV-associated neurocognitive disorders (HAND), which affects over 1.5 million Africans yearly. While incidence of HAND has decreased with the use of highly active antiretroviral therapy, prevalence has increased due to longer life expectancy. Other NeuroAIDS syndromes include tuberculous meningitis and intracerebral tuberculoma, cryptococcal meningitis, toxoplasma encephalitis, progressive multifocal leukoencephalopathy, primary central nervous system lymphoma, stroke, and distal sensory polyneuropathy. NeuroAIDS care and research in Africa are hindered by resource limitations. Inadequate neuroimaging and laboratory facilities result in diagnostic delays and confusion, while limited access to drugs leads to inappropriate treatment. However, the situation may be improving. Better funding of HIV care by African governments and donor agencies have resulted in decreasing HIV prevalence and prolonged survival. Yet, central nervous system opportunistic infections remain important causes of death and disability among African patients with HIV/AIDS. There is the need for additional funding to improve access to antibiotics and to facilitate further research into NeuroAIDS and its treatment.

Stroke risk factors, subtypes, and 30-day case fatality in Abuja, Nigeria.

BACKGROUND: Stroke is the second leading cause of death and the leading cause of adult disability worldwide. A better understanding of stroke risk factors and outcome may help guide efforts at reducing the community burden of stroke. This study aimed to understand stroke risk factors, imaging subtypes, and 30-day outcomes among adult Nigerians. MATERIALS AND METHODS: We prospectively recruited all patients presenting with acute stroke at the National Hospital Abuja between January 2010 and June 2012. We assessed clinical and laboratory variables, as well as brain computerized tomography, magnetic resonance imaging, and carotid Doppler ultrasound scans. We also assessed case fatality and functional outcome at 30 days after stroke. RESULTS: Of 272 patients studied, 168 (61.8%) were males. Age at presentation (mean ± standard deviation) was 56.4 ± 12.7 years in males and 52.9 ± 14.8 years in females (P = 0.039). Neuroimaging was obtained in 96.7% patients, revealing cerebral infarction (61.8%), intracerebral hemorrhage (ICH) (34.8%), and subarachnoid hemorrhage (SAH) (3.4%). Carotid plaques or stenosis ≥50% were detected in 53.2% patients with cerebral infarction. Stroke risk factors included hypertension (82.7%), obesity (32.6%), diabetes (23.5%), hyperlipidemia (18.4%), atrial fibrillation (9.2%), and cigarette smoking (7.7%). At 30 days after stroke, case-fatality rate was 18.8%, whereas modified Rankin Scale (mRS) scores for cerebral infarction, ICH, and SAH were 3.71, 4.21, and 4.56, respectively. Atrial fibrillation, a previous stroke, and age older than 50 years were all associated with worse mRS scores at 30 days. CONCLUSION: Although hypertension, obesity, diabetes mellitus, and atrial fibrillation were important stroke risk factors, in many patients, these were detected only after a stroke. While the commonest stroke subtype was cerebral infarction, observed in almost two-third of patients, SAH was associated with the highest case-fatality rate at 30 days of 44.4%. Larger population-based studies may provide additional data on stroke incidence and outcome among Nigerians.