Jérôme Jobe

Intravenous immunoglobulins in paraneoplastic brainstem encephalitis with anti-Ri antibodies

Sirs: We report the case of a 71 year old man who developed over 2 days severe cerebellar ataxia, dysarthria, dysphagia, opsoclonus-myoclonus, nausea and vomiting. Brain MRI was normal. The cerebrospinal fluid (CSF) contained 39 lymphocytes/ mm3 with normal protein and glucose levels. An extensive biological investigation remained negative except for the presence of anti-Ri antibodies. These anti-Ri antibodies were demonstrated by a combination of immunofluorescence analysis and Western blot (anti-neuronal antibody Western blot immunoassay, Immco Diagnostics Inc., Buffalo, NY). Serial dilutions revealed a specific antibody titre of 1 : 320. Thoracic CT revealed a suspect nodular mass in the right lung (middle lobe). Bronchoscopy with broncho-alveolar wash was normal. A full-body PET was planned but due to the severity of dysphagia and the fact that patient became bedridden, intravenous immunoglobulins (IVIg, 400 mg/kg/day for 5 days) were administered 4 weeks after symptom onset. This treatment induced a clear-cut clinical improvement after 1 week, allowing removal of the nasogastric tube and a restart of oral feeding. The patient could walk with some aid. Retest for anti-Ri antibodies was negative. The PET scan was in favour of a right lung neoplasm. Unfortunately, the patient developed a pyelonephritis with septicaemia and died 8 weeks after the onset of symptoms. Autopsy confirmed a diagnosis of lung adenocarcinoma. In paraneoplastic neurological disorders (PNDs), the most frequently found antineuronal antibodies are anti-Hu, anti-Yo and anti-CRMP5; anti-Ri antibodies are rare. In 1988 and 1991, BuddeSteffen et al. [2] and Luque et al. [6] described a subpopulation of patients with opsoclonus and a history of breast cancer, in sera and CSF in whom they identified an antibody they namedAnti-Ri. After those seminal reports, anti-Ri antibodies have subsequently been associated with a broad spectrum of malignancies, the most frequent being breast, gynaecological and lung cancers [8] and their spectrum of associated neurological symptoms has also expanded considerably. In patients with anti-Ri antibodies, antitumour treatment is recommended as it can also act on paraneoplastic symptoms [10]. Few data are available over efficacy of immunotherapy in anti-Ri syndromes. Shams’ili et al. [10], Rajabally et al. [9] and Martinaud et al. [7] reported some experience with the use of IVIg (3 patients treated) with opposite issues. Our case illustrates the possibility of rapid onset of PNDs. A paraneoplastic syndrome should be considered in cases of subacute brainstem encephalitis. Moreover, despite the fatal outcome, this observation highlights the therapeutic potential of IVIg in neurological paraneoplastic disorders, with clinical improvement, albeit transient and the hypothesis of a neutralization of anti-Ri antibodies (of which this is the first case report). One could postulate that any immunotherapy might have had the same efficacy as IVIg in our patient. Indeed, various immunomodulatory approaches used so far in PND have brought striking improvement but only in single patients (See Voltz [14] for a review). For example, treatment options that have been tried include protein A absorption [3], intravenous immunoglobulin [1, 12], cyclophosphamide [5, 11, 13], methylprednisolone [5, 15] and plasma exchanges [4, 13]. Owing to the equivocal efficacy of such treatments, it seems up to now difficult to outline general guidelines in PND for the use of immunotherapy which should probably be adapted to the individual patient’s situation. Further studies are needed to determine which patients with PND (characterized by antineuronal antibodies and clinical syndromes) could benefit from which immunotherapy. One must keep in mind that the earlier immunotherapy is started the better the chances of improvement [1, 5]. We thank ZLB Belgium for providing Sandoglobuline as compassionate treatment for this patient.

A fatal case of Perthes syndrome

Perthes syndrome, or traumatic asphyxia, is a clinical syndrome associating cervicofacial cyanosis with cutaneous petechial haemorrhages and subconjonctival bleeding resulting from severe sudden compressive chest trauma. Deep inspiration and a Valsalva maneuver just prior to rapid and severe chest compression, are responsible for the development of this syndrome. Current treatment is symptomatic: urgent relief of chest compression and cardiopulmonary resuscitation if needed. Outcome may be satisfactory depending on the duration and severity of compression. Prolonged thoracic compression may sometimes lead to cerebral anoxia, irreversible neurologic damage and death. We report a fatal case of Perthes syndrome resulting from an industrial accident.

Quantifying emergency department crowding: comparison between two scores

Abstract Introduction: Emergency department (ED) crowding is a major international concern with a negative impact on both patient care and providers. Currently, there is no consensus regarding measure of crowding. Therefore, emergency physicians have to choose between numerous scoring systems, from simple to more complex. The aim of the present study was to compare the complex National Emergency Department Overcrowding Scale (NEDOCS) with the simple ED Occupancy rate (OR) determination. We further evaluated the correlation between these scores and a qualitative assessment of crowding. Method: This study was conducted in two academic and one regional hospital in Liege Province, in Belgium; each accounting for an ED census of over 40,000 patient visits per year. Crowding measures were sampled four times a day, over a two-week period, in January 2016. Results: ED staff considered overcrowding as a major concern in the three ED. Median OR ranged from 68 to 100, while NEDOCS ranged from 64.5 to 76.3. A significant correlation was found in each ED between the OR and the NEDOCS (Pearson r = 0.973, 0.974 and 0.972), as well as between the OR, the NEDOCS and the subjective evaluation by the ED staff (p = 0.001). Conclusion: Crowding evaluation in ED requires validated scores. Our study in three different hospitals demonstrates that simple OR appeared as accurate as more sophisticated NEDOCS. Furthermore, this measure is perfectly correlated with the feeling of ED staff.