The relationship between obstructive sleep apnea and Parkinson's disease: a systematic review and meta-analysis.

BACKGROUND: Obstructive sleep apnea (OSA) is a common sleep disorder in Parkinson's disease (PD). However, the relationship between OSA and PD is still inconsistent. Our study was aimed to evaluate the relationship between PD and OSA. METHODS: Studies on OSA and PD were searched using PubMed, Embase, Web of Science, Cochrane library, and Chinese National Knowledge Infrastructure databases. Review Manager 5.3 software was used to calculate the pooled estimate effect. The inverse variance model was used to pool the mean difference (MD) or hazard ratios (HRs); the Mantel-Haenszel method was used to pool the odds ratio (OR). Heterogeneity among the studies was assessed using I2 statistic and Q test. RESULTS: A total of 12 studies with 93,332 cases were deemed eligible and included in our meta-analysis. Overall, the occurrence of PD was more frequent in patients with OSA (HR 1.59, 95% CI, 1.36-1.85). The subgroup analysis demonstrated the risk similarly by sex. Male and female had HR of incident PD with OSA of 1.56 (95% CI, 1.30-1.87) and 1.60 (95% CI, 1.21-2.11), respectively. The incidence of OSA did not increase in PD patients (OR 0.89, 95% CI, 0.53-1.49). The MD of apnea-hypopnea index (AHI) in PD patients was also not statistically significant (P = 0.5). CONCLUSIONS: The results indicate that OSA is one of independent risk factors of PD. However, OSA does not seem to be abnormally frequent in PD.

Coexistent Charcot-Marie-Tooth type 1A and type 2 diabetes mellitus neuropathies in a Chinese family.

Charcot-Marie-Tooth disease type 1A (CMT1A) is caused by duplication of the peripheral myelin protein 22 (PMP22) gene on chromosome 17. It is the most common inherited demyelinating neuropathy. Type 2 diabetes mellitus is a common metabolic disorder that frequently causes predominantly sensory neuropathy. In this study, we report the occurrence of CMT1A in a Chinese family affected by type 2 diabetes mellitus. In this family, seven individuals had duplication of the PMP22 gene, although only four had clinical features of polyneuropathy. All CMT1A patients with a clinical phenotype also presented with type 2 diabetes mellitus. The other three individuals had no signs of CMT1A or type 2 diabetes mellitus. We believe that there may be a genetic link between these two diseases.

Carbamazepine Withdrawal-induced Hyperalgesia in Chronic Neuropathic Pain.

Combined pharmacological treatments are the most used approach for neuropathic pain. Carbamazepine, an antiepileptic agent, is generally used as a third-line treatment for neuropathic pain and can be considered an option only when patients have not responded to the first- and second-line medications. In the case presented herein, a patient with neuropathic pain was treated using a combined pharmacological regimen. The patient's pain deteriorated, despite increasing the doses of opioids, when carbamazepine was discontinued, potentially because carbamazepine withdrawal disrupted the balance that was achieved by the multifaceted pharmacological regimen, thus inducing hyperalgesia. Interestingly, when carbamazepine was prescribed again, the patient's pain was successfully managed. Animal research has reported that carbamazepine can potentiate the analgesic effectiveness of morphine in rodent models of neuropathic pain and postoperative pain. This clinical case demonstrates that carbamazepine may have a synergistic effect on the analgesic effectiveness of morphine and may inhibit or postpone opioid-induced hyperalgesia. We postulate that a probable mechanism of action of carbamazepine may involve -aminobutyric acid-ergic potentiation and the interruption of glutamatergic function via N-methyl-D-aspartate receptors. Further research is warranted to clarify the analgesic action of carbamazepine and its potential use for the prevention of opioid-induced hyperalgesia in chronic neuropathic pain patients.

Nerve biopsy findings contribute to diagnosis of multiple mononeuropathy: 78% of findings support clinical diagnosis.

Multiple mononeuropathy is an unusual form of peripheral neuropathy involving two or more nerve trunks. It is a syndrome with many different causes. We reviewed the clinical, electrophysiological and nerve biopsy findings of 14 patients who suffered from multiple mononeuropathy in our clinic between January 2009 and June 2013. Patients were diagnosed with vasculitic neuropathy (n = 6), perineuritis (n = 2), chronic inflammatory demyelinating polyradiculoneuropathy (n = 2) or Lewis-Sumner syndrome (n = 1) on the basis of clinical features, laboratory data, electrophysiological investigations and nerve biopsies. Two patients who were clinically diagnosed with vasculitic neuropathy and one patient who was clinically diagnosed with chronic inflammatory demyelinating polyradiculoneuropathy were not confirmed by nerve biopsy. Nerve biopsies confirmed clinical diagnosis in 78.6% of the patients (11/14). Nerve biopsy pathological diagnosis is crucial to the etiological diagnosis of multiple mononeuropathy.

Intraneural perineurioma affecting multiple nerves: a case report and literature review.

Intraneural perineurioma is a neoplasm of perineurial cells, corresponding to WHO grade I. We present a case of intraneural perineurioma affecting multiple nerves, which usually involved one or two of major nerve trunks in one patient. We describe the clinical presentation, magnetic resonance (MR) neurography characteristics, and pathological characteristics. The differential diagnosis with other diseases, such as neurofibroma, Schwannomatosis and HNPP, will also be discussed. We also review the literature in efforts to highlight recent studies on intraneural perineurioma and heighten and awareness for the possible presentations of this disorder.

[Significance of Nogo-A expression of atrophic muscle fibers in diagnosis of amyotrophic lateral sclerosis].

OBJECTIVE: To investigate significance of Nogo-A in atrophic muscle fibers in diagnosis of amyotrophic lateral sclerosis (ALS). METHODS: Forty cases which were diagnosed definitely by clinical, pathological or DNA analysis were included. All of the cases underwent muscle biopsies in order to carry out Nogo-A immunostaining. RESULTS: Nogo-A expression was detected in the atrophic muscle fibers but in either neurogenic disease or myogenic disease, the atrophic muscle fibers demonstrated expression of Nogo-A. As compared with the stainings of NADH-TR and ATPase, it was showed that Nogo-A positive fibers were mainly type I fibers. CONCLUSION: Our results show that the presence of Nogo-A in diseased human muscle biopsies is not limited to ALS, therefore it cannot be the standard for ALS diagnosis.

[The correlation of CAGs repeat size with age of onset in patients with Kennedy's disease].

OBJECTIVE: To investigate the correlation of CAGs repeat size and age of onset in patients with Kennedy's Disease (KD). METHODS: We detected the number of CAG repeats in the androgen receptor genes in 30 patients with KD. The correlation of CAGs repeat size with age of onset was analyzed. At the same time, the Appel scale that could represent the degree of motor functional impairment was scored in every patient. The correlation of Appel scale with CAGs repeat size and the course of disease were analyzed. RESULTS: Significant correlation was found between the number of CAGs with age of onset (r = -0.671, P < 0.01). There was also correlation between the Appel score and the course of disease (r = 0.855, P < 0.01), but no correlation between the Appel score and the number of CAGs (r = 0.100, P = 0.601). CONCLUSIONS: It is found that in Kennedy' disease, as well as in other CAG repeat diseases, the length of polyglutamine tract determines the age of onset, but has no correlation with the severity of the disease.

[The relationship between carotid artery atherosclerosis and hyperhomocysteinaemia].

OBJECTIVE: To explore the relationship between the carotid artery atherosclerosis (CAA) and the level of homocysteine (Hcy) as well as folate, vitamin B(12) and lipids. METHODS: The diameter of common carotid arteries and internal carotid arteries on both sides were measured by B-mode ultrasound in 126 subjects who were divided into normal and A, B, C, D groups according to the severity of stenosis of CAA. With fasting serum, Hcy as well as folate, vitamin B(12), triglycerides, total cholesterols (TC), low density lipid (LDL), high density lipid (HDL) were detected. RESULTS: When stenosis of CAA became severer, serum Hcy was higher. The levels of the normal and A, B, C, D groups were (13.22 +/- 6.15, 16.29 +/- 9.81, 19.49 +/- 11.16, 27.21 +/- 17.47, 24.14 +/- 8.64) micromol/L, respectively. Rank test showed a significant difference between the normal and other groups (P < 0.01). Folic acid and VitB(12) were negatively correlated with the Hcy concentrations. Spearman correlation coefficient were -0.23 and -0.42 (P = 0.000). According to the severity of stenosis of CAA, Hcy, age, weight, blood pressure, level of glucose, triglyceride, TC, LDL, high density lipid (HDL) and history of hypertension, diabetes, heart disease and stroke were brought into logistic analysis; the result showed that Hcy, TC, HDL had significant negative correlation with the severity of CAA, Spearman correlation coefficient were 0.56, 0.25 and 0.22 (P = 0.003, 0.02, 0.04). CONCLUSION: Hyperhomocysteinaemia is an independent risk factor of CAA and the severity of stenosis of CAA is highly correlated with Hcy concentration. The causes of hyperhomocysteinaemia may be the results of decrease of folate and VitB(12) levels. Clinical trials are now required to evaluate the effect of treatment with these vitamins in the primary and secondary prevention of vascular diseases.

[Establishment of the organotypic model of amyotrophic lateral sclerosis from the SD rats' spinal cord].

OBJECTIVE: To establish an in vitro model of amyotrophic lateral sclerosis (ALS) from the organotypic culture of SD rats' lumber spinal cord induced by the mitochondrial inhibitor,malonate sodium. METHOD: The lumber spinal cord prepared from the 6-day-old SD rats was cut into 350 microm coronarily, cultured on the Millicell-CM inserts which make the spinal cord culturing on the interface between air and fluid. First, the optimum malonate sodium dose was determined by adding different doses into the medium and counting the living motor neuron numbers by using immuno-histochemistry staining. Second, the ALS model was established as following: the cultures were divided into the malonate groups and the control groups, adding 2 mmol/L sodium malonate into the medium of the malonate groups an the 3rd day, continue culture to 12 days with this concentration; the control groups culturing without malonate. RESULTS: The organotypic characteristics are still kept till the end of the curlturing. After adding the sodium malonate, counting the number of motor neurons and interneurons on the different spinal slices in the different groups, the number of motor neuron in the cultured spinal cord was less than control (11.00+/-2.45 vs 15.29+/-1.70 per semislice at the end of the culturing, P<0.01), but the difference of the interneuron was not significant. CONCLUSION: The amyotrophic lateral sclerosis model is successful with selective injury of motor neuron, and this model can be used for the exploring of the theraptic method and its pathogenesis.

Immunohistochemical characterisation of pulmonary hyaline membrane in various types of interstitial pneumonia.

AIMS: Hyaline membrane (HM) in diffuse alveolar damage (DAD) pattern is frequently detected in the acute stage of interstitial pneumonia (IP). To determine the exact nature of HM, we investigated immunohistochemically 25 cases of HM-containing IP. METHODS: The cases examined using various kinds of antibodies were four cases associated with rheumatoid arthritis, five with usual interstitial pneumonia, two with dermatomyositis, five with viral infection, one case with progressive systemic sclerosis and eight cases caused by other agents. RESULTS: HM mostly reacted with antibodies to PE10 (SP-A), Factor VIII, KL-6 and EMA and, interestingly, stained for AE1/AE3, CK19, and Hup-1 in some cases, but was negative for PTAH staining. However, the immunoreactivities of HM varied even within the same disease or section. CONCLUSIONS: The immunohistochemical heterogeneity of HM suggests that HM may be formed by different mechanisms in various types of IP. Our findings also suggest that the main components of HM are derived from alveolar epithelial cells and their proteins, some including cytoplasmic element of CK19, and also from serum factors, but not fibrin. The immunohistochemical characteristics of HM in DAD pattern will aid understanding of the significance of HM formation in IP.

Typical nemaline bodies presenting in a patient with polymyositis.

A 66-year-old woman presented with a progressive myopathy affecting the proximal limbs and unusual pathological findings of nemaline bodies on muscle biopsy. Histological examination demonstrated that the bodies were mainly located in the subsarcolemmal region of atrophic fibers, exhibited strong immunoreactivity with antibodies to both alpha-actinin and m-actin, and had a typical lattice-like appearance at higher magnification on electron microscopy. These findings were the same as those for nemaline myopathy. The patient responded to steroid therapy, but relapse occurred after steroid was discontinued. Given the clinical criteria of polymyositis, we believe that the occurrence of nemaline bodies in our patient should be interpreted primarily as an epiphenomenon of primary myopathy.