Wernicke encephalopathy after subtotal gastrectomy for morbid obesity

Wernicke's encephalopathy (WE) is one of the potential complications of obesity surgery. It is an acute neuropsychiatric syndrome resulting from thiamine deficiency often associated with repeated vomiting. The classic triad is frequently reported in these patients (optic neuropathy, ataxia and confusion), associated with uncommon features. Cerebral impairment affects the dorsal medial nucleus of the thalamus and the periaqueductal grey area, appearing on MRI, as hyperintense signals on T2, Flair and Diffusion weighted imaging. Early diagnosis and parenteral thiamine are required to decrease morbidity and mortality. We report a case of WE and Korsakoff's syndrome in a young obese patient after subtotal gastrectomy, who still has substantial sequelae. The contribution of MRI with diffusion-weighted imaging is illustrated. The interest of nutritional supervision in the first weeks and preventive thiamine supplementation in case of repeated vomiting are of particular importance in these risky situations.     

EFNS guidelines for diagnosis,therapy and prevention of Wernicke encephalopathy

Background: Although Wernicke encephalopathy (WE) is a preventable and treatable disease it still often remains undiagnosed during life. Objectives: To create practical guidelines for diagnosis, management and prevention of the disease. Methods: We searched MEDLINE, EMBASE, LILACS, Cochrane Library. Conclusions and recommendations:

• 1 The clinical diagnosis of WE should take into account the different presentations of clinical signs between alcoholics and non alcoholics (Recommendation Level C); although prevalence is higher in alcoholics, WE should be suspected in all clinical conditions which could lead to thiamine deficiency (good practice point – GPP).
• 2 The clinical diagnosis of WE in alcoholics requires two of the following four signs; (i) dietary deficiencies (ii) eye signs, (iii) cerebellar dysfunction, and (iv) either an altered mental state or mild memory impairment (Level B).
• 3 Total thiamine in blood sample should be measured immediately before its administration (GPP).
• 4 MRI should be used to support the diagnosis of acute WE both in alcoholics and non alcoholics (Level B).
• 5 Thiamine is indicated for the treatment of suspected or manifest WE. It should be given, before any carbohydrate, 200 mg thrice daily, preferably intravenously (Level C).
• 6 The overall safety of thiamine is very good (Level B).
• 7 After bariatric surgery we recommend follow‐up of thiamine status for at least 6 months (Level B) and parenteral thiamine supplementation (GPP).
• 8 Parenteral thiamine should be given to all at‐risk subjects admitted to the Emergency Room (GPP).
• 9 Patients dying from symptoms suggesting WE should have an autopsy (GPP).

     

Neurologic outcome of choreoathetoid encephalopathy after cardiac surgery

Intractable seizures remain a significant therapeutic challenge despite current advances in the treatment of epilepsy. Thyrotropin-releasing hormone, the first neuroendocrine releasing factor to be isolated and fully characterized, was also the first releasing factor investigated as a possible neurotransmitter/neuromodulator outside the hypothalamus. Basic and clinical research has revealed a distinct neuroanatomic distribution and a neurochemical role for thyrotropin-releasing hormone in seizure modulation. Thyrotropin-releasing hormone and selected analogs were reported to have antiepileptic effects in several animal seizure paradigms, including kindling and electroconvulsive shock. Clinically, thyrotropin-releasing hormone treatment has been reported to be efficacious in such intractable epilepsies as infantile spasms, Lennox-Gastaut syndrome, myoclonic seizures, and other generalized and refractory partial seizures. Herein, we review evidence that suggests that thyrotropin-releasing hormone and selected thyrotropin-releasing hormone analogs may represent a new class of novel antiepileptic drugs, namely, antiepileptic neuropeptides and provide insights into potential new treatments for the intractable epilepsies.     

Acute ophthalmoplegia and nystagmus in infants fed a thiamine-deficient formula: an epidemic of Wernicke encephalopathy.

In 2003, an epidemic of Wernicke encephalopathy (WE) developed in Israeli infants fed a thiamine-deficient soy-based formula. Approximately 20 infants were affected out of an estimated 3500 fed the vitamin-deficient formula. The finding of gaze abnormalities in a single infant by neuro-ophthalmologists led to the unraveling of the epidemic. In this report, the findings in three infants are described. Early diagnosis and treatment with parenteral thiamine led to complete neurologic recovery in two infants; in the third infant, delayed diagnosis may have been responsible for severe lingering deficits. This is the first reported epidemic of WE secondary to thiamine-deficient infant formula. Early diagnosis and treatment are critical to avoid persistent neurologic impairment.     

Post-operative hematoma after surgery for intracranial meningiomas: causes, avoidable risk factors and clinical outcome

Intracranial meningiomas are mainly benign lesions amenable for surgical resection. However, removal of an intracranial meningioma carries a higher risk of post-operative hemorrhage compared to surgery for other intracranial neoplasms. Because avoidance of post-operative hematoma is of vital interest for neurosurgical patients, the aim of this retrospective study was to analyze risk factors of post-operative hematoma associated with meningioma surgery. Two hundred and ninety six patients with intracranial meningiomas, operated between June 1998 and June 2002, were included in this study. Patients who developed a space-occupying post-operative intracranial hemorrhage and were treated surgically were identified. Data of patients with and without hematoma were retrospectively analyzed to identify risk factors associated with post-operative hematoma. Variables analyzed included patients' age, invasion of venous sinus by the meningioma, tumor vascularization, arachnoidal infiltration, pre-operative prophylaxis of thromboembolic events, peri-operative coagulation abnormalities, residual tumor, location and histology of the tumor. Outcome of patients with post-operative hematoma was assessed according to the Glasgow Outcome Scale (GOS) at discharge and at three months. 21 patients (7.1 %) of 296 patients developed a post-operative intracranial hematoma requiring surgical evacuation. Age was significantly higher in the hematoma group 62.4 +/- 14.0 years compared to patients without post-operative hematoma 56.1 +/- 12.0 (p < 0.05; t-test). Patients older than 70 years had a six-fold increased risk to develop a post-operative hematoma (Chi2 test, 95% CI 1.949-13.224). Patients with post-operative hemorrhage had significant lower post-operative prothrombin time, fibrinogen and platelets immediately after surgery and lower platelets at day 1. None of the other parameters, including pre-operative routine coagulation values, differed significantly between patients with and without post-operative hemorrhage. Three patients with post-operative hematoma showed platelet dysfunction and three patients showed decreased FXIII activity. Of those patients with post-operative hemorrhage at three months follow up three patients (13%) succumbed from reasons not directly related to hemorrhage, one patient remained GOS 2 (4.3%), four patients (17.4%) were GOS 3 and 15 (65.4%) patients had favorable outcome (GOS 4 [one patient] and GOS5 [14 patients]). Meningioma surgery carries a higher risk for post-operative hematoma in the elderly. Thrombocytopenia and other hemostatic disorders were frequently associated with post-operative hemorrhage after meningioma surgery, while no surgical factors could be defined. Extending coagulation tests and specific replacement therapy may prevent hematoma formation and improve the patients outcome.     

Medical genetics diagnostic evaluation of the child with global developmental delay or intellectual disability

PURPOSE OF REVIEW: The review addresses the recent discovery of large-scale copy number variations in the human genome and advances in microarray technology which together have changed the clinical genetic diagnostic approach for children with global developmental delay. RECENT FINDINGS: Several publications in the last three years evaluate the diagnostic rate of array comparative genomic hybridization (aCGH) in the setting of global developmental delay; to date, the rate of etiologic diagnosis ranges from 4.8% to 20%, a potential doubling of the rate of diagnosis prior to the application of this technique. SUMMARY: Large-scale copy number variations in the human genome leading to gene dosage imbalances comprise approximately 12% of the entire genome and some 10% of all known genes. Molecular testing for chromosome imbalances has changed with the application of array comparative genomic hybridization. Recent publications suggest a doubling of the rate of diagnosis of patients with genome copy number abnormalities as the cause of developmental delay. The use of array comparative genomic hybridization is replacing the use of fluorescent in-situ hybridization techniques for the child with idiopathic global developmental delay or intellectual disability.     

Clinical outcome with gamma-knife surgery or surgery for brain metastases from colorectal cancer

The aim of this study was to investigate the clinical outcomes after gamma knife surgery (GKS) or surgery as the first treatment for brain metastases in colorectal cancer (CRC). Of the 4350 patients diagnosed with CRC at our institution identified from 1987 to 2009, 27 patients who underwent GKS (GKS group) and 11 who underwent surgery (surgery group) were included. The oncologic outcomes were compared between the two groups. Local control was significantly better in the surgery group than in the GKS group (90% versus [vs.] 71.4%, respectively; p = 0.006). The rate of symptom relief after 3 months was significantly higher in the surgery group than in the GKS group (72.7 vs.18.5%, respectively; p = 0.005). The median survival after GKS was 5.6 months and surgery was 16.2 months. In multivariate analysis, controlled primary tumor (p = 0.038) and solitary metastasis (p = 0.028) were correlated with prolonged overall survival, whereas surgery (p = 0.034) was associated with longer local control. Surgery for brain metastasis from CRC is more advantageous in local control and neurologic symptom palliation than GSK. In multivariate analysis, overall survival was associated with controlled primary tumor and solitary metastasis.     

Temporal lobe epilepsy surgery: different surgical strategies after a non-invasive diagnostic protocol

AIM: To test a non-invasive presurgical protocol for temporal lobe epilepsy (TLE) based on "anatomo-electro-clinical correlations". METHODS: All consecutive patients with suspected TLE and seizure history <2 years were entered into the protocol, which included video-electroencephalographic (EEG) monitoring and magnetic resonance imaging (MRI). Three different TLE subsyndromes (mesial, lateral, mesiolateral) were identified by combined anatomical, electrical, and clinical criteria. "Tailored" surgery for each subsyndrome was offered. Patients with seizure history <2 years, MRI evidence of temporal mass lesion, and concordant interictal EEG and clinical data bypassed video-EEG monitoring and were directly scheduled for surgery. RESULTS: Lesionectomy was performed without video-EEG recording in 11 patients with tumorous TLE. Of 146 patients studied with video-EEG, 133 received a TLE diagnosis. Four were excluded for neuropsychological risks, eight refused surgery, and 121 underwent surgery. Of 132 consecutive patients who underwent surgery, 101 had at least one year of follow up. They were divided into a "hippocampal sclerosis/cryptogenic" group (n = 57) and a "tumours/cortical organisation disorders" group (n = 44). In the first group, extensive temporal lobectomy (ETL) was performed in 40 patients, anteromesial temporal lobectomy (AMTL) in 17 patients. At follow up, 47 patients were seizure free. In the second group, lesionectomy plus ETL was performed in 23 patients, lesionectomy plus AMTL in six patients, and lesionectomy alone in 15 patients. Thirty nine patients were seizure free. CONCLUSIONS: These findings suggest that different TLE subsyndromes can be identified accurately using non-invasive anatomo-electro-clinical data and can be treated effectively and safely with tailored surgery.     

Psychological distress at follow-up after major surgery for intra-oral cancer

A cohort of patients who had received major intra-oral surgery, up to 10 yr (mean 3.5 yr) prior to this study were reviewed and assessed for evidence of current psychological disturbance. Forty-one per cent of patients were found to have significant levels of psychological distress comprising mood disorder, social dysfunction or both types of difficulty. Prevalence of clinical anxiety and depression per se was within the range 22-32%, but few patients were receiving any form of active therapy for these problems. Investigation of predictors revealed that females and younger people were more at risk to psychological distress. Tentative evidence for variability in psychological outcome in relation to tumour site is also reported. Results are discussed with reference to service provision needs and further research priorities.