Superficial Siderosis.

Herein, we describe a case of 69 year old man who presented with gradual bilateral hearing impairment and unsteadiness. Examination showed bilateral sensorineural deafness and cerebellar ataxia. Neuroimaging revealed a rim of hypointensity surrounding the brain stem, superficial cerebellar fissures and extending into the cervical spinal cord consistent with the diagnosis of superficial siderosis (SS). Investigations failed to identify any reversible cause for the condition.

Is it important to classify ischaemic stroke?

Thirty-five percent of all ischemic events remain classified as cryptogenic. This study was conducted to ascertain the accuracy of diagnosis of ischaemic stroke based on information given in the medical notes. It was tested by applying the clinical information to the (TOAST) criteria. Hundred and five patients presented with acute stroke between Jan-Jun 2007. Data was collected on 90 patients. Male to female ratio was 39:51 with age range of 47-93 years. Sixty (67%) patients had total/partial anterior circulation stroke; 5 (5.6%) had a lacunar stroke and in 25 (28%) the mechanism of stroke could not be identified. Four (4.4%) patients with small vessel disease were anticoagulated; 5 (5.6%) with atrial fibrillation received antiplatelet therapy and 2 (2.2%) patients with atrial fibrillation underwent CEA. This study revealed deficiencies in the clinical assessment of patients and treatment was not tailored to the mechanism of stroke in some patients.

The clinical spectrum of Lyme neuroborreliosis.

Lyme disease is a multisystem infectious disease, endemic in parts of Europe, including the West of Ireland. Neurological manifestions (neuroborreliosis) are variable. Presenting neurological syndromes include meningitis, cranial neuropathies, myeloradiculitis and mononeuritis multiplex. A lack of specificity in serological diagnosis may add to diagnostic confusion. We reviewed thirty cases of acute Lyme disease in the West of Ireland and found neurological syndromes in 15 (50%), with painful radiculopathy (12 patients; 80%) and cranial neuropathy (7 patients; 46%) occurring frequently. Neuroborreliosis needs to be considered in the differential diagnosis of these neurological syndromes in the appropriate clinical context.

When a stroke is not a stroke; posterior leukoencephalopathy syndrome mimicking posterior circulation stroke.

Posterior leukoencephalopathy syndrome (PLS) is a potentially reversible syndrome that may mimic the clinical and radiological features of posterior circulation cerebral infarction. Three cases of PLS are presented which were erroneously diagnosed as strokes and treated in accordance with recent evidence based guidelines; none of the cases fulfilled the current criteria requiring treatment for hypertension in the acute stroke setting. Once the diagnosis of PLS was made, and the patients blood pressure treated aggressively, all patients had rapid and full clinical resolution of their symptoms. Given the important differences in management and prognosis, rapid and accurate diagnosis is essential. Posterior leukoencephalopathy syndrome needs to be considered in patients presenting with clinical and/or radiological findings that predominantly affect the occipital lobes.

Should we perform elective inguinal hernia repair in the elderly?

PURPOSE: Many surgeons are reluctant to offer elective inguinal and femoral hernia repair (IHR) to the elderly due to concerns of increased risk. The authors sought to evaluate the outcomes of elderly patients undergoing IHR compared to the general population. METHODS: We performed a retrospective review of the 2011 NSQIP database evaluating 19,683 patients undergoing IHR. Patients were divided by age into three categories: <65, 65-79 and >80. Logistic regression analysis was used to assess impact of comorbid conditions and type of surgery on outcomes. Patients were analyzed for mortality and complications based on their age and the types of surgery (elective, urgent, emergent, laparoscopic versus open) and comorbid conditions. RESULTS: There were 17,375 male patients (88 %). 92.7 % were elective. 70 % were performed using an open technique. Age distribution was 63.4 % < 65, 26.6 % 65-79, 10 % >80. Mortality was similar across age groups in elective repair. Mortality was increased in emergency repair in all age groups (p < 0.001). Mortality was increased in emergency surgery compared to elective surgery in patients >80 (OR = 57, p < 0.001). Mortality was similar between laparoscopic and open in <65 (OR = 0.96, p = 0.97) and unable to be assessed in other age groups. Dyspnea and COPD predicted higher mortality and complications with emergency surgery in the elderly (age 65-79 OR 15.3 and 14.9, respectively, age >80 OR 56.5 and 14.9, respectively). CONCLUSIONS: Elective inguinal hernia repair carries a similar mortality in the elderly compared to the general population. Emergent IHR carries a very high risk of death in the elderly. The authors recommend considering elective IHR regardless of age.

Radiological remission and recovery of thirst appreciation after infliximab therapy in adipsic diabetes insipidus secondary to neurosarcoidosis.

BACKGROUND: Neurosarcoidosis is a rare and aggressive variant of systemic sarcoidosis which may result in hypothalamic-pituitary dysfunction. We report a case of hypothalamic hypopituitarism secondary to neurosarcoidosis complicated by adipsic diabetes insipidus (ADI). Initiation of anti-tumour necrosis factor-α (TNF-α) therapy resulted in both radiological disease remission and recovery of osmoregulated thirst appreciation after 3 months. CASE SUMMARY: A 22-year-old man was referred to the endocrinology service with profound weight gain, polyuria and lethargy. Biochemical testing confirmed anterior hypopituitarism while posterior pituitary failure was confirmed by hypotonic polyuria responding to desmopressin. Magnetic resonance imaging (MRI) demonstrated extensive hypothalamic infiltration; neurosarcoidosis was confirmed histologically after excisional cervical lymph node biopsy. Osmoregulated thirst appreciation was normal early in the disease course despite severe hypotonic polyuria. However, subsequent subjective loss of thirst appreciation and development of severe hypernatraemia in the setting of normal cognitive function indicated onset of ADI. MANAGEMENT: Clinical management involved daily weighing, regular plasma sodium measurement, fixed daily fluid intake and oral desmopressin. We initiated immunosuppressive therapy with pulsed intravenous anti-TNF-α therapy (infliximab) after multidisciplinary team consultation. OUTCOME: Infliximab therapy resulted in successful radiological disease remission and complete recovery of osmoregulated thirst appreciation. This was confirmed by subjective return of thirst response and maintenance of plasma sodium in the normal range in the absence of close biochemical monitoring.

Expression of N-methyl-D-aspartate receptor subunit mRNAs in the human brain: striatum and globus pallidus.

N-methyl-D-aspartate receptors (NRs) play an important role in basal ganglia function. By using in situ hybridization with ribonucleotide probes, we investigated the regional and cellular distribution of NR subunit mRNA expression in the human basal ganglia: caudate nucleus, putamen, lateral globus pallidus (LGP), and medial globus pallidus (MGP). Analysis of both film autoradiograms and emulsion-dipped slides revealed distinct distribution patterns for each subunit. On film autoradiograms, the signal for NR1, NR2B, and NR2C in the striatum (STR) was higher than in globus pallidus (GP). The NR2D probe gave a stronger signal in GP than in STR. For NR2A we found a signal in all regions. Analysis of emulsion-dipped sections demonstrated that in striatal neurons, the NR2B signal was higher than in GP neurons. In GP neurons, NR2D was more abundant than in striatal neurons. Despite the relatively low signal on film for NR2C in GP, we found a slightly higher signal in GP per neuron than in STR since in the pallidal areas neurons were sparse but intensely labeled. NR1 and NR2A were more evenly distributed over neurons of STR and GP Between the different parts of STR and GP, we observed only minor differences in the expression of NRs. In MGP a subpopulation of neurons exhibiting low NR2D signals could be separated from the majority of neurons showing an intense NR2D signal. Since the physiological properties of NRs are dependent on subunit composition, these data suggest a high degree of regional specialization of NR properties in the human basal ganglia.

Expression of N-methyl-D-aspartate receptor subunit mRNAs in the human brain: hippocampus and cortex.

N-methyl-D-aspartate receptor (NR) activation in the hippocampus and neocortex plays a central role in memory and cognitive function. We analyzed the cellular expression of the five NR subunit (NR1 and NR2A-D) mRNAs in these regions with in situ hybridization and human ribonucleotide probes. Film autoradiograms demonstrated a distinct pattern of hybridization signal in the hippocampal complex and the neocortex with probes for NR1, NR2A, and NR2B mRNA. NR2C and NR2D probes yielded scattered signals without a distinct organization. At the emulsion level, the NR1 probe produced high-density hybridization signals across the hippocampal complex. NR2A mRNA was higher in dentate granule cells and pyramidal cells in CA1 and subiculum compared to hilus neurons. NR2B mRNA expression was moderate throughout, with higher expression in dentate granule cells, CA1 and CA3 pyramidal cells than in hilus neurons. In the hippocampal complex, the NR2C probe signal was not different from background in any region, whereas the NR2D probe signal resulted in low to moderate grain densities. We analyzed NR subunit mRNA expression in the prefrontal, parietal, primary visual, and motor cortices. All areas displayed strong NR1 hybridization signals. NR2A and NR2B mRNAs were expressed in cortical areas and layers. NR2C mRNA was expressed at low levels in distinct layers that differed by region and the NR2D signal was equally moderate throughout all regions. Pyramidal cells in both hippocampus and neocortex express NR1, NR2A, NR2B, and, to a lesser extent, NR2D mRNA. Interneurons or granular layer neurons and some glial cells express NR2C mRNA.

Expression of N-methyl-D-aspartate receptor subunit mRNA in the human brain: mesencephalic dopaminergic neurons.

Evidence is accumulating that glutamate-mediated excitotoxicity plays an important role in neuronal degeneration in Parkinson's disease (PD). In addition, alterations in excitatory amino acid neurotransmission in the basal ganglia contribute to the clinical manifestations of motor dysfunction. However, detailed knowledge of the anatomical distribution and subtype specificity of glutamate receptors in the dopamine neurons of human substantia nigra (SN) has been lacking. In order to test the hypothesis that selective expression of particular N-methyl-D-aspartate receptor (NR) subunit mRNA contributes to the differential vulnerability of specific neuronal populations to excitotoxic injury in PD, we have used a quantitative dual label, in situ hybridization technique with ribonucleotide probes to examine the cellular distribution of NR subunit mRNA in postmortem human mesencephalic dopaminergic neurons from subjects with no known neurological disorder. Analysis of both film autoradiograms and emulsion-dipped sections demonstrated significant labeling of nigral neurons for each NR subunit. Neuronal labeling was most intense for the NR1 and NR2D subunits, with low level labeling for the remaining subunits. In addition, we examined four subregions of the ventral mesencephalon for differential expression of NR subunit mRNA. For all NR subunits, the pars lateralis (PL) exhibited the most intense signal, while neurons of the ventral tier substantia nigra pars compacta (SNpc) failed to demonstrate a preponderance of a particular subunit. These results demonstrate that NRs are expressed to a significant degree in dopaminergic neurons of the SN and that their distribution does not correlate with the characteristic pattern of neuronal degeneration in PD.

Outcomes following staged bilateral pallidotomy in advanced Parkinson's disease.

The authors assessed clinical outcome for up to one year after staged bilateral pallidotomy in 14 patients with advanced PD. One year after surgery, dyskinesias were virtually abolished and there were significant reductions in "off" time (67%) and activities of daily living "off" scores (24%), as well as nonsignificant reduction in "off" motor score (39%); "on" scores were unchanged. One patient developed a visual field deficit; two had transient confusion. Staged bilateral pallidotomy improves motor function in selected patients with advanced PD.

Effects of diuretics on the renal handling of magnesium.

Diuretic-induced magnesium losses may contribute significantly to magnesium deficiency. Loop diuretics especially cause major losses of urinary magnesium. Our initial experiments in saline-loaded rats indicated that frusemide greatly increased magnesium excretion, while bendrofluazide caused no significant change and triamterene actually decreased magnesium excretion. Therefore, not all diuretics are magnesium-wasting. There is evidence that the potassium-sparing diuretic amiloride may also exert magnesium-sparing properties. In saline-loaded rats, the magnesium-sparing effect of amiloride was demonstrated when the drug was administered either alone or in combination with frusemide. Renal clearance studies in rats indicated that the magnesium-sparing effect of amiloride was a direct renal action and not secondary to possible extrarenal actions. A dose-response relationship has been established for the effect of amiloride in reducing fractional excretion of magnesium and potassium during frusemide diuresis in rats. Congestive heart failure patients being treated with frusemide were found to be both potassium- and magnesium-deficient, as indicated by reduced lymphocyte concentrations of potassium and magnesium. In these patients, amiloride reduced urinary magnesium and potassium, increased plasma magnesium and potassium, and also increased lymphocyte magnesium and potassium. The effects of amiloride most likely involve enhanced reabsorption of magnesium, but further studies are required to establish the precise mechanism(s) involved in the drug's magnesium-sparing properties.

Experience with endoluminal colonic wall stents for the management of large bowel obstruction for benign and malignant disease.

HYPOTHESIS: To assess the applicability and efficacy of endoluminal colonic wall stents (ECWSs) in the management of large bowel obstruction (LBO). DESIGN: Inception cohort study. SETTING: University-based tertiary medical center. PATIENTS: Eleven consecutive patients with LBO in the absence of peritonitis. INTERVENTION: Placement of ECWS under endoscopic and fluoroscopic guidance. MAIN OUTCOME MEASURES: The success rate in ECWS placement, the efficacy in decompressing the obstruction, and the patency rate of the ECWS. RESULTS: Successful placement of ECWSs was obtainable in 10 of 11 patients. Once placed, all 10 patients achieved immediate decompression of their LBO. Eight patients had malignant obstructions associated with distant spread of disease; 3 patients had diverticular disease. Among those with malignant obstruction, 6 patients had successful and lasting palliation without colostomy, 1 patient underwent 1-stage resection 1 month later with no evidence of obstruction, and 1 patient could not be stented so diversion was done. None of the patients with diverticular disease required diversion: 2 had complete bowel preparation followed by resection with primary anastomosis, whereas the third declined surgery. Four of the 10 patients required overlapping ECWSs to bridge the stricture. One patient required a second ECWS secondary to recurrence of obstruction after stent migration and has continued palliation of his stage 4 rectal cancer for the last 11 months. No other complications were encountered. CONCLUSIONS: Urgent surgery with colostomy for LBO was avoided in 10 of 11 patients because of successful placement of ECWSs. We believe that endoscopic colonic stenting is safe, effective, and lasting, and should be considered as initial nonoperative management in all patients seen with LBO in the absence of peritonitis.

Stereotactic pallidotomy performed without using microelectrode guidance in patients with Parkinson's disease: surgical technique and 2-year results.

OBJECT: Pallidotomy for the treatment of medically refractory Parkinson's disease (PD) has enjoyed renewed popularity. However, the optimal surgical technique, lesion location, and long-term effectiveness of pallidotomy remain subjects of debate. In this article the authors describe their surgical technique for performing pallidotomy without using microelectrode guidance, and the clinical and radiological results of this procedure. METHODS: Patients were evaluated preoperatively by using a battery of validated clinical rating scales and magnetic resonance (MR) imaging of the brain. Individuals with severe treatment-refractory idiopathic PD who were believed to be good candidates for surgery underwent computerized tomography scanning- and MR imaging-guided stereotactic pallidotomy. Intraoperative macrostimulation was used to optimize lesion placement and to avoid injury to nearby structures. Lesion location and size were calculated from MR imaging sequences of the brain obtained within the first 24 hours after surgery and again 3 months later. Clinical examinations were conducted at 1.5, 3, 6, 12, and 24 months after surgery. Seventy-five patients (mean age 61 years, range 38-79 years) underwent unilateral pallidotomy. Significant improvements were observed in the "off' period scores for the activities of daily living portion of the Unified Parkinson's Disease Rating Scale (UPDRS), the UPDRS motor scores, total "on" time, levodopa-induced dyskinesias, and contralateral tremor. These improvements were maintained 24 months postoperatively. The mean lesion volume measured on the immediate postoperative MR image was 73 +/- 5.4 mm3. Radiological analysis suggests that initial lesion volume does not predict outcome. The only permanent major complication was a single visual field defect. CONCLUSIONS: Pallidotomy performed without using microelectrode guidance is a safe and effective treatment for selected patients with medically refractory PD.

Pseudomembranous colitis.

Pseudomembranous colitis is an inflammatory disease of the colon and rectum characterized by the development of elevated mucosal plaques. It usually is associated with antibiotic therapy and is caused by elaboration of toxin from the anaerobic bacterium, Clostridium difficile. The hallmark of treatment is orally administered vancomycin or metronidazole. The mortality rate is high in patients whose condition is not diagnosed and appropriately treated. Emergency surgery occasionally is needed for complications, including colonic perforation and toxic colitis.

Acute cholecystitis resulting from hemobilia after tru-cut biopsy: a case report and brief review of the literature.

Acute cholecystitis following hemobilia is very rare, with only five cases reported in the literature. A case report of a 22-year-old woman who underwent a liver biopsy for staging of Hodgkin's lymphoma and developed cholecystitis due to hemobilia is presented. The incidence of hemobilia has increased with the advent of more invasive hepatobiliary procedures, but the mortality has been decreasing due to better recognition and therapy. Acute cholecystitis associated with hemobilia is very rare, but will be seen with increasing frequency; and a high index of suspicion needs to be maintained to ensure timely diagnosis and treatment.

Independent predictors of morbidity and mortality in blunt colon trauma.

We sought to determine the impact of (1) grade of the colon injury, (2) the formation of an ostomy, and (3) associated injuries on outcomes such as morbidity and mortality after blunt colon injuries. We retrospectively reviewed 16,814 cases of blunt abdominal trauma. Patients with colonic injuries were selected and charts reviewed for demographic, clinical, and outcomes data. Injuries were grouped by the Colon Injury Scale (grades I-V). Independent risk factors of morbidity included spine and lung injuries, as well as increased age. A higher grade of colon injury trended toward a significant association with intra-abdominal complications. Independent risk factors of mortality included liver, heart, and lung injuries, as well as intracerebral blood and female gender. The grade of colon injury, the formation of an ostomy, and management of the colon trauma did not independently predict increased intra-abdominal complications, morbidity, or mortality. These results indicate that patients afflicted with blunt colon trauma experience a high rate of morbidity and mortality from associated injuries and or increased age. Treatment regimens directed at these factors will be most helpful in reducing the high morbidity and mortality after blunt colon trauma. Factors such as ostomy formation and management strategy are not associated with increased morbidity or mortality after blunt colon trauma.

Necrotizing enterocolitis in a neonatal piglet model.

The aim of this study was to develop an animal model for necrotizing enterocolitis (NEC). Twenty-five neonatal Hanford minipigs had carotid artery and external jugular vein catheters and rectal Clinical Tonomitors placed under anesthesia. Experimental animals were subjected to a hypoxic insult (50% reduction in baseline PaO2 for 30 minutes) and hypothermic stress (core temperature reduced to 35 degrees C for 30 minutes). Regular oral diet was resumed and the survivors were euthanized 3 to 4 days later. All animals underwent necropsy with gross and histopathological evaluation of the entire bowel. Of 22 experimental animals, 14 survived (64%) and 8 (36%) died of pulmonary hemorrhage. Of the 14 survivors, 8 (57%) had gross and microscopic evidence of NEC. Six of the total 25 animals (24%) sustained rectal perforations from the tonometer. Of 3 control animals, one died of pulmonary hemorrhage and the two survivors had normal intestine. This model successfully produced gross and histological evidence of NEC. The tonometer shows promise as a predictor of NEC provided technical modifications can reduce the complication rate.

Captopril for refractory hypertension in patients with impaired renal function.

The use of captopril in 19 patients with renal parenchymal disease and refractory hypertension was studied for a mean period of 12 months. There was a significant reduction in the systolic and diastolic blood pressures, with a reduction in the mean arterial pressure of 29 mmHg. The mean maintenance dose of captopril was 142 mg daily in three divided doses. All but one of the patients required a diuretic for satisfactory blood pressure control and 3 patients were also given a beta-blocker. In all patients a simplification of the previous therapeutic regimen was achieved. A significant rise in serum creatinine was noted in 2 patients, one of whom had to be withdrawn from the study. Despite the presence of renal functional impairment, proteinuria did not occur de novo nor did established proteinuria increase. Leukopenia was noted in any of the patients in this group.

Does long-term partial sodium channel blockade alter disease progression in MS? Evidence from a retrospective study.

BACKGROUND: There is accumulating evidence that long-term disability and disease progression in multiple sclerosis (MS) are due to prolonged sodium channel opening along demyelinated axons. Despite good evidence in animal models of MS that partial voltage-gated sodium channel (VGSC) blockade reduces disease progression, little is known about its effects in patients, despite widespread use of such agents in the symptomatic management of MS. OBJECTIVE: To determine if long-term exposure to the VGSC-blocking drug carbamazepine (CBZ) alters disease progression in MS. METHODS: Using a retrospective chart review of patients diagnosed with MS, we compared progression of disability between patients exposed the VGSC blocker CBZ with those who were not exposed to the drug. Both whole-group and matched case-control analyses were performed after correcting for the influence of age, gender, MS subtype, expanded disability status score at diagnosis, use of disease-modifying therapy, and year of initial therapy. The multiple sclerosis severity scale (MSSS) was used as a measure of disease severity. The primary outcome measure was MSSS score difference between groups. RESULTS: Four hundred patients were included; 51 received CBZ symptomatic therapy (average duration of therapy 27 months). There was no significant difference in mean MSSS between the two groups in either the whole group comparison (p = 0.63) or the matched analysis (p = 0.12). CONCLUSION: Despite preclinical evidence suggesting a neuroprotective role of VGSC blockers in animal models of MS, this retrospective study suggests that long-term exposure to the VGSC-blocking drug CBZ fails to alter long-term disability and disease progression in MS patients.