Cushing Syndrome is Associated with Increased Stage N2 Sleep and Decreased SWS Partially Reversible After Treatment.

The aim of the present study was to evaluate the sleep parameters of patients with Cushing syndrome (CS) at the time of diagnosis and 12-months after treatment. Thirty four newly diagnosed patients with endogenous CS (17 with ACTH-secreting pituitary adenoma, 17 with adrenal CS) and 23 controls with similar age were included in the study. Two polysomnography (PSG) recordings were performed; one at the time of diagnosis and the other 12 months after resolution of hypercortisolemia. Control group had only baseline PSG. Based on the PSG findings, stage N2 sleep was found to be prolonged, stage N3 and REM sleep were shortened in patients with CS. Average heart rate and mean Apnea Hypopnea Index (AHI) score were higher in patients with CS than the control subjects. Sixteen (47.1%) patients with CS and 4 (17.4%) controls had obstructive sleep apnea (OSA; AHI ≥5). There were no significant differences in sleep parameters of patients according to the etiology of CS (adrenal vs. pituitary) patients. Following 12-months of treatment, a significant decrease in stage N2 sleep and a significant increase in stage N3 sleep were detected, but there was no change in terms of AHI. In conclusion, Cushing syndrome has disturbing effects on sleep structure and these effects are at least partially reversible after treatment. However, the increased risk of OSA was not reversed a year after treatment indicating the importance of early diagnosis and treatment of CS.

Obstructive sleep apnoea prevalence in non-arteritic anterior ischaemic optic neuropathy.

AIMS: The aim of this study was to show the prevalence of obstructive sleep apnoea (OSA) in non-arteritic anterior ischaemic optic neuropathy (NAION). METHODS: 20 patients diagnosed with NAION were included in the study. 20 age and sex matched subjects with similar risk factors for NAION, such as diabetes mellitus (DM) and hypertension (HT), constituted the control group. All cases underwent polysomnography for investigation of the presence of OSA. Cases with an Apnoea-Hypopnoea Index >5 were accepted as having OSA. RESULTS: Mean ages of the patients and controls were 60.90±8.14 and 61.15±7.23 years, respectively. There were no significant differences between the patient and control groups in terms of age, gender, body mass index, smoking/alcohol consumption or systemic diseases. In the patient group, 85% were diagnosed with OSA compared with 65% in the control group (p>0.05). CONCLUSIONS: We found a high prevalence of OSA in patients with NAION but it was also high in the control group (p>0.05). This may be due to the fact that the two groups were matched for the same risk factors for NAION. The study indicates that OSA is not a risk factor for NAION in itself but is the contributing factor as it has effects on the vascular endothelium in DM, HT and atherosclerosis.

Refractory generalized seizures as a possible side effect of bevacizumab in a colon cancer patient.

Bevacizumab, which is a humanized monoclonal antibody against vascular endothelial growth factor, is used to treat metastatic cancers of the colon. Adverse effects common with bevacizumab treatment are hypertension, arterial-venous thrombosis, bleeding, gastrointestinal perforation, and proteinuria. To date, there have been no reports of refractory seizure following treatment with bevacizumab. We describe a patient who presented with refractory generalized tonic-clonic seizures after receiving last dose of bevacizumab for treatment of metastatic colorectal cancer with FOLFIRI and bevacizumab regimen.

Sleep architecture in Sheehan's syndrome before and 6 months after growth hormone replacement therapy.

OBJECTIVE: To characterize the sleep parameters in patients with growth hormone (GH) deficiency in Sheehan's syndrome adults and to assess the effects of 6-month GH replacement therapy (GHRT). METHODS: Twenty-two women with Sheehan's syndrome, (mean age; 49.1+/-2.2 years), and 12 women with similar age (mean age; 51.3+/-3.8 years) and body mass index as control subjects were included in the study. Under baseline conditions, women received adequate hormone replacement therapy for all hormonal deficiencies other than GH. Twelve patients received recombinant GH (Genotropin; Pfizer Stockholm, Sweden) (treatment group) and eight patients received placebo (placebo group) for 6 months. Two patients had only baseline evaluation and were not followed up prospectively. Two polysomnography (PSG) recordings were performed on the patients group, one in the baseline period and the other at the sixth month of treatment (either GH or placebo). Control group had only baseline PSG. RESULTS: GH deficient females with Sheehan's syndrome have more NREM (95.9+/-1.5% and 88.6+/-0.9%, respectively; p<0.05), particularly in stage 4 sleep (11.4+/-1.9% and 4.9+/-1.6, respectively; p<0.05), less REM sleep (4.2+/-1.5% and 11.4+/-0.9, respectively; p<0.05) and also less sleep efficiency (69.7+/-3.4% and 81.1+/-2.8%, respectively; p<0.05) when compared to healthy controls. After 6 months of GHRT there was no significant difference in sleep parameters. CONCLUSION: GH deficiency has sleep disturbing effects on Sheehan's syndrome patients under baseline conditions.

Therapeutic plasma exchange in patients with neurologic diseases: retrospective multicenter study.

Therapeutic plasma exchange (TPE) is commonly used in many neurological disorders where an immune etiology was known or suspected. We report our experience with TPE performed for neuroimmunologic disorders at four university hospitals. The study was a retrospective review of the medical records of neurological patients (n=57) consecutively treated with TPE between April 2006 and May 2007. TPE indications in neurological diseases included Guillain-Barrè Syndrome (GBS) (n=41), myasthenia gravis (MG) (n=11), acute disseminated encephalomyelitis (ADEM) (n=3), chronic inflammatory demyelinating polyneuropathy (CIDP) (n=1) and multiple sclerosis (MS) (n=1). Patient median age was 49; there was a predominance of males. Twenty-two patients had a history of other therapy including intravenous immunoglobulin (IVIG), steroid, azothioprin, and pridostigmine prior to TPE. Another 35 patients had not received any treatment prior to TPE. All patients were classified according to the Hughes functional grading scores pre- and first day post-TPE for early clinical evaluation of patients. The TPE was carried out 1-1.5 times at the predicted plasma volume every other day. Two hundred and ninety-four procedures were performed on 57 patients. The median number of TPE sessions per patient was five, and the median processed plasma volume was 3075mL for each cycle. Although the pre-TPE median Hughes score of all patients was 4, it had decreased to grade 1 after TPE. While the pre-TPE median Hughes score for GBS and MG patients was 4, post-TPE scores were decreased to grade 1. Additionally, there was a statistically significant difference between post-TPE Hughes score for GBS patients with TPE as front line therapy and patients receiving IVIG as front line therapy (1 vs. 3.5; p=0.034). Although there was no post-TPE improvement in Hughes scores in patients with ADEM and CIDP, patients with MS had an improved Hughes score from 4 to 1. Mild and manageable complications such as hypotension and hypocalcemia were also observed. TPE may be preferable for controlling symptoms of neuroimmunological disorders in early stage of the disease, especially with GBS.

Different seizure types and skin lesions in oculocerebrorenal syndrome of Lowe.

Oculocerebrorenal syndrome of Lowe is an X-linked recessive disorder localized to Xq24-26.1. The phenotypic features of this disorder are Fanconi-type renal failure, mental retardation, and various eye abnormalities. Seizures may accompany the disease, and the skin-related findings are poorly defined. This case of a 9-year-old patient, diagnosed as having and followed for oculocerebrorenal syndrome of Lowe, has been presented for his seizures, which were initially myoclonic but subsequently atonic, and for his skin findings, understood to be trichoepithelioma, cystic in nature, and stemming from mature hair follicles. In monitoring the disease, the manifestation of the seizures as atonic seizures accompanied by focally initiated secondary generalized epileptic discharges is a finding previously undefined in oculocerebrorenal syndrome of Lowe. Moreover, the presence of dermal findings of a cystic nature is reported in few cases of this syndrome. In this rare syndrome, it is necessary to be aware of the presence of atonic seizures, which have an association with the progression of the disease that has not been previously reported in the literature, and of the cystic dermal lesions as part of the syndrome.