Diagnostic imaging in 13 cases of Rasmussen's encephalitis: can early MRI suggest the diagnosis?

Rasmussen's encephalitis (RE) is a rare, progressive, chronic encephalitis characterised by drug-resistant epilepsy, progressive hemiparesis and mental impairment. It typically involves only one cerebral hemisphere, which becomes atrophic. We present neuroradiological findings in 13 children with RE. MRI was performed in all patients, fluorodeoxyglucose positron-emission tomography (PET) in three, Tc-99m hexamethylpropylenamine oxime single-photon emission computed tomography (SPECT) in two and proton MR spectroscopy ((1)HMRS) in two. MRI showed progression of the hemisphere atrophy, always prevalent in the region primarily involved (13 patients), spread of the abnormal signal in white matter (11) and cortex (10) and progression of atrophy of the head of the caudate nucleus (nine). Associated secondary changes were: atrophy of the contralateral cerebellar hemisphere (in four patients), the ipsilateral hippocampus (in five) and the brain stem (in five). The earliest CT and MRI abnormalities, seen between 1 day and 4 months after the first seizure (in 12 patients examined, nine of whom had MRI) in one cerebral hemisphere included: high signal on T2-weighted images in the cortex (seven patients) and white matter (nine), cortical atrophy usually involving the frontoinsular region, with mild or severe enlargement of the lateral ventricle (eight) and moderate atrophy of the head of the caudate nucleus (seven). Cortical swelling in the early stage of the disease was recognisable only in two patients. PET revealed hypometabolism, SPECT decreased perfusion, and (1)HMRS reduction of N-acetylaspartate in the affected hemisphere. PET and SPECT were usually performed in the late stages and did not provide specific findings. MRI thus demonstrates the progression of RE and may suggest the diagnosis in the early stages, often before the appearance of neurological deficits. Early diagnosis of RE may be crucial for selecting patients for aggressive medical therapy or major surgical interventions such as hemispherectomy.     

Necrotizing periodontitis as a possible manifestation of common variable immunodeficiency

Common variable immunodeficiency (CVID) is an inherited disease characterized by hypogammaglobulinaemia and impaired humoural immunoresponse and is mainly associated with recurrent infections of the airway and the digestive tract. An 18-year old female with a diagnosis of CVID associated with a devastating necrotizing periodontitis, ultimately resulting in complete destruction of the periodontium and loss of all teeth, is reported. Clinical, biochemical, microbiological and radiographic examinations are presented. The report highlights the likely importance of immunoglobulin replacement and intensive dental hygiene in CVID patients, and the devastating effect of non-compliance in such patients.     

Acute heart failure following severe chloralose poisoning: A case report

Self-poisoning with alpha chloralose is frequent in Tunisia. Neurological signs are prominent involving myoclonic jerks and coma. Cardiac toxicity is not well known.We report the case of a 19 year-old woman who ingested a chloralose rodenticide and was admitted to our intensive care unit. Four hours after admission she developed acute heart failure with pulmonary edema and elevated troponin. Echocardiography showed diffuse hypokinesia. Patient’s cardiac and respiratory status improved progressively and echocardiography performed 5 days after was normal.Cardiac toxicity following acute chloralose poisoning is uncommon and potentially serious. Its mechanism is poorly understood.     

Rasch-built Overall Disability Scale for patients with chemotherapy-induced peripheral neuropathy (CIPN-R-ODS)

Chemotherapy-induced peripheral neuropathy (CIPN) is a common neurological side-effect of cancer treatment and may lead to declines in patients’ daily functioning and quality of life. To date, there are no modern clinimetrically well-evaluated outcome measures available to assess disability in CIPN patients. The objective of the study was to develop an interval-weighted scale to capture activity limitations and participation restrictions in CIPN patients using the Rasch methodology and to determine its validity and reliability properties. A preliminary Rasch-built Overall Disability Scale (pre-R-ODS) comprising 146 items was assessed twice (interval: 2–3 weeks; test–retest reliability) in 281 CIPN patients with a stable clinical condition. The obtained data were subjected to Rasch analyses to determine whether model expectations would be met, and if necessarily, adaptations were made to obtain proper model fit (internal validity). External validity was obtained by correlating the CIPN-R-ODS with the National Cancer Institute-Common Toxicity Criteria (NCI-CTC) neuropathy scales and the Pain-Intensity Numeric-Rating-Scale (PI-NRS).The preliminary R-ODS did not meet Rasch model’s expectations. Items displaying misfit statistics, disordered thresholds, item bias or local dependency were systematically removed. The final CIPN-R-ODS consisting of 28 items fulfilled all the model’s expectations with proper validity and reliability, and was unidimensional.The final CIPN-R-ODS is a Rasch-built disease-specific, interval measure suitable to detect disability in CIPN patients and bypasses the shortcomings of classical test theory ordinal-based measures. Its use is recommended in future clinical trials in CIPN.     

Near real-time echocardiography teleconsultation using low bandwidth and MPEG-4 compression: feasibility, image adequacy and clinical implications

We assessed the feasibility, image adequacy and clinical utility of a tele-echocardiography service which combined video compression with low-bandwidth store-and-forward transmission. Echocardiograms were acquired by a hospital geriatrician, compressed and transmitted using both near real-time (urgent) and delayed (pre-programmed) protocols via an Internet connection to the notebook PC of a remote cardiologist. Clinical utility was evaluated as a change in therapeutic management. During a one-year period, 101 tele-echocardiography consultations were successfully performed (feasibility = 100%) on 95 patients (age 22-95 years), admitted with cardiovascular or neurological diagnoses (24% of the consultations were urgent). In total, 4617 files (1.4 GByte of data) were transmitted, 2669 of which were short video clips. On average, 46 files (13.8 MByte) were transmitted (mean duration 10 min) at each examination. Consultations (both urgent and pre-programmed) were clinically useful in 83% of examinations. Logistic regression analysis showed that both a low left ventricular systolic function and the examination indication were determinants of clinical utility. The transmitted images were considered adequate for diagnosis in 100% of the pre-programmed teleconsultations. Tele-echocardiography using MPEG-4 video compression is a feasible, adequate and clinically useful tool for telemedicine.