Primary angiitis of CNS : neuropathological study of three autopsied cases with brief review of literature

Primary angiitis of CNS(PACNS) or granulomatous angiitis of CNS is a rare inflammatory disease of small blood vessels mostly confined to the CNS. The clinical and pathological features of 3 autopsied cases are described. Clinically all the three PACNS patients were young males, age ranging from 19 to 31 years. All presented with varied neurological manifestations. There was no evidence of systemic disease in any of the cases. The ESR was normal and CSF analysis showed chronic meningitic pattern. The cerebral angiogram in one case was normal and the CT scan done in another case showed multiple intracerebral haematoma due to vasculitis. Brain biopsy was not done. Diagnosis was made at post-mortem examination. Histology showed characteristic but variable degree of granulomatous and non-granulomatous angiitis of small vessels. Venulitis with parenchymal haemorrhages was the predominant feature and in one case phlebitis with thrombosis was noted. Since the disease responds to steroids and immunosuppressive therapy, establishing antemortem diagnosis is important. In view of the association of angiitis of CNS with bacteria and viral infections, their role in the evolution of the disease needs to be investigated.     

小剂量胰岛素联合电解质补充治疗儿童糖尿病酮症酸中毒的临床价值研究

目的：研究小剂量胰岛素联合电解质补充治疗儿童糖尿病酮症酸中毒的临床疗效。方法以该院2012年5月-2014年5月收治的30例糖尿病酮症酸中毒患儿为研究对象,均接受小剂量胰岛素、电解质补充等治疗,统计患儿酸中毒纠正时间、住院时间,同时比较治疗前后患儿血糖、钠、钾水平及主要血清炎症因子变化。结果治疗后患儿病情均稳定,平均酸中毒纠正时间、平均住院时间分别为(13.50±1.12)、(14.00±1.05)d。治疗前后患儿血糖[(38.66±2.37)VS(12.07±2.85)]mmol/L、血钾[(5.52±0.81)VS(4.70±0.78)]mmol/L、血钠[(133.27±10.41)VS(142.68±9.98)]mmol/L比较差异有统计学意义(P<0.01)。另外,治疗前后IL-6、IL-12、IL-18及TNF-α水平比较差异有统计学意义(P<0.01)。结论小剂量胰岛素联合电解质补充治疗能明显改善患儿高血糖、高血酮症状,安全有效,可作为儿童糖尿病酮症酸中毒治疗的重要手段。     

PMP22 Gene–Associated Neuropathies: Phenotypic Spectrum in a Cohort from India

Journal of Molecular Neuroscience - Reports of spectrum of clinical manifestations in PMP22 gene–associated neuropathies (duplication/mutations) are scarce. To identify the frequency of PMP22...     

Absence of age-related changes in nigral dopaminergic neurons of Asian Indians: Relevance to lower incidence of Parkinson's disease

Age-related loss of melanized nigral neurons reported in the British Caucasians is not observed in Asian Indian, American and French adults. In the Americans, loss of dopaminergic phenotype occurs from midlife, without frank neurodegeneration. Here, we investigated whether nigral dopaminergic neurons in Asian Indians are lost with age or undergo morphological or biochemical dysfunction. Using unbiased stereology we estimated volume, number of melanized, borderline/non-melanized (n=34, 28 gestational weeks to 80 years) and tyrosine hydroxylase (TH)–Nurr1 co-labeled neurons (n=32, 28 gestational weeks to 80 years) in substantia nigra pars compacta. We quantified Nurr1 and TH proteins by immunoblotting (n=18, 28 gestational weeks to 69 years) and apoptotic neurons by terminal deoxynucleotidyl transferase mediated dUTP nick end labeling (TUNEL) staining. Nuclear and soma size was estimated by morphometry. There was no age-related decline in volume, neuronal density, neuronal numbers and TH-Nurr1 co-labeled neurons. TH and Nurr1 protein expression remained stable. Lack of TUNEL-TH co-labeled cells confirmed absence of neuronal apoptosis. The neuronal size remained unaltered. Our findings of preserved nigral dopaminergic neurons suggest no age-related loss of nigral function in Asian Indians, unlike the Americans. This may explain the lower incidence of Parkinson's disease in Asian Indians.     

Brain biopsy in Creutzfeldt-Jakob disease: evolution of pathological changes by prion protein immunohistochemistry

The formation of protease-resistant prion protein (PrPsc) is considered to be an early event in the pathogenesis of Creutzfeldt-Jakob disease (CJD) and hence its demonstration in brain biopsies by immunohistochemistry is considered diagnostic. We analysed eight brain biopsies from the frontal cortex collected from different parts of India from cases diagnosed as CJD on clinical and pathological grounds for the expression of prion protein (PrP). The duration of illness in these cases varied from 2 months to 1 year. Immunohistochemistry was carried out on paraffin sections using two different clones (KG9 and 3F4) of monoclonal antibodies to PrP. Although all eight cases showed classical features of spongiform encephalopathy of varying severity, only five of the eight cases revealed PrP(sc) in the brain tissue. The immunolabelling was focal and all areas with spongiform change were not labelled. A temporal evolution in the staining pattern was evident - particulate diffuse labelling (synaptic type) in early stages (2 months), perivacuolar deposits in intermediate stages (5-6 months), and dense plaques in late stages (12 months).     

Inclusion body myositis (IBM)

Clinical, histological, immunohistochemical and ultrastructural features of 5 cases of inclusion body myositis -4 sporadic (s-IBM) and one hereditary (h-IBM) form are described. These patients (3 men, 2 women) had chronic progressive weakness of varying severity in all 4 extremities with sparing of cranial muscles. Elevation of CPK was noted in 2 patients. Electromyography revealed features of myopathy in 4 and additional neurogenic changes in 2 subjects. Clinical diagnosis was often other than inclusion body myositis. Presence of characteristic eosinophilic inclusions within the vacuoles established the diagnosis. The inclusions were congophilic and showed positivity to ubiquitin, beta-amyloid and SMI-31 in the sporadic cases while congophila was absent in the hereditary form. Immunostaining to hyperphosphorylated-tau was negative in both s-IBM and h-IBM. Membraneous whorls were observed at ultrastructural level. None of the patients improved with steroids and trial with other immunosuppressants was unsuccessful.     

Vasculitic neuropathy in HIV infection: a clinicopathological study

Vasculitis causing peripheral neuropathy may be the first sign of HIV infection. We report four such cases in whom the onset of peripheral neuropathy led to the detection of HIV infection. Two patients presented with features of mononeuritis multiplex, while the other two had a lumbosacral polyradiculopathy. A prior history of blood transfusion was forthcoming in one of the patients. Sural nerve biopsies in all the four cases and the muscle biopsy in two, histologically showed evidence of vasculitis. Immunohistochemically, the viral antigen was not demonstrable in any of the biopsies, but on electron microscope, virus-like particles were identifiable in the Schwann cell cytoplasm and the perivascular macrophages in one case. To the best of our knowledge, this is the only report that has documented the virus in the Schwann cells as well as the perivascular macrophages lending credence to the fact that these viruses are neurotropic as well as lymphotropic. Immunoglobulin deposits were not demonstrable in any of the cases, suggesting that direct viral invasion may have a role in the pathogenesis of peripheral nerve vasculitis.     

Cerebral zygomycosis

Fifty-six patients with cerebral zygomycosis (mucormycosis) were seen during the period 1971-2001 in two tertiary care hospitals located in south India with tropical climate and catering to neurological diseases. Forty-four patients had rhinocerebral and twelve patients had isolated central nervous system (CNS) zygomycosis. Of these, ten were culture proven (Rhizopus oryzae in eight and Mucor in two); 30 were diagnosed as probable and 16 were diagnosed possible; mixed infections were seen in three patients. Diabetes mellitus was the predisposing condition in a majority (31/44) of patients with the rhinocerebral form of zygomycosis. The tissue obtained at biopsy/autopsy in either form showed necrotic/infarcted tissue with neutrophilic infiltration with broad non-septate hyphae showing irregular branching. The outcome was poor despite surgical excision and antifungal therapy. The high concentration of spores in a mouldy environment, the bird population and improper disposal of hospital waste may facilitate healthy hosts presenting with primary CNS disease.     

Outcomes of carotid angioplasty and stenting for radiation-associated stenosis

BACKGROUND AND PURPOSE: In light of their high surgical risk, carotid angioplasty and stent placement may be preferred in patients with radiation-associated carotid stenosis. The purpose of this study was to determine the procedural complication rate, patency, and clinical outcomes after carotid angioplasty and stent placement in this small group of high-risk patients. METHODS: Sixteen patients (mean age, 65 years; 5 women and 11 men) who received radiation therapy for head and/or neck malignancy subsequently developed carotid stenosis (mean, 84%; range, 70%-99%) in a total of 19 carotid arteries, which were treated with angioplasty and stent placement. The patients were followed for a mean time of 28 months (range, 5-78 months) with periodic Doppler studies, angiography, CT angiography, or clinically. RESULTS: In the total 19 stented carotid arteries, 23 procedures were performed (22 stent placement procedures and one repeat angioplasty). The procedural stroke rate was 1/23 (4%). The procedural transient ischemic attack rate was 0/23 (0%). There was one other observed complication: a puncture site hematoma. The 30-day postprocedure complication rate was 0/23 (0%); no neurologic symptoms were reported. Fifteen of the 19 vessels (79%) developed no new stenosis throughout the follow-up period. Two of 19 (11%) vessels had repeat angioplasty and stent placement; 1/19 (5%) had a repeat angioplasty. One restented vessel has remained patent for 50 months. Another restented vessel required a third stent placement 17 months after the second. Two of 19 (11%) vessels occluded per Doppler examination 14 and 22 months postprocedure. CONCLUSION: Angioplasty and stent placement have low rates of complications and restenosis in the treatment of radiation-associated carotid occlusive disease.