We report a patient who presented with severe cold-induced allodynia and hyperhidrosis, and found to have acquired neuromyotonia (Isaacs syndrome) with high voltage-gated potassium channel (VGKC) antibody titre,positive contactin-associated protein 2 (CASPR2) and leucine-rich glioma-inactivated 1 (LGI1) antibodies. The patient also had positive anti-dsDNA and acetylcholine receptor (AChR) antibodies without clinical features of SLE or myasthenia gravis, suggesting a strong underlying autoimmune tendency. CT thorax showed no thymoma. Her symptoms improved with intravenous immunoglobulin infusion but recurred despite maintenance oral corticosteroids and carbamazepine. She has since been on regular IVIG infusions. Cold allodynia is an unusual presentation in acquired neuromyotonia.
We report a fatal case of post-partum streptococcal toxic shock syndrome in a patient who was previously healthy and had presented to the emergency department with an extensive blistering ecchymotic lesions over her right buttock and thigh associated with severe pain. The pregnancy had been uncomplicated, and the mode of delivery had been spontaneous vaginal delivery with an episiotomy. She was found to have septicemic shock requiring high inotropic support. Subsequently, she was treated for necrotizing fasciitis, complicated by septicemic shock and multiple organ failures. A consensus was reached for extensive wound debridement to remove the source of infection; however, this approach was abandoned due to the patient's hemodynamic instability and the extremely high risks of surgery. Both the high vaginal swab and blister fluid culture revealed Group A beta hemolytic streptococcus infection. Intravenous carbapenem in combination with clindamycin was given. Other strategies attempted for streptococcal toxic removal included continuous veno-venous hemofiltration and administration of intravenous immunoglobulin. Unfortunately, the patient's condition worsened, and she succumbed to death on day 7 of hospitalization.
Children with Down syndrome have a higher risk of stroke. Similarly, intravenous immunoglobulin (IV Ig) is also known to cause a stroke. We reported a 3-year-old boy with Down syndrome who presented with severe pneumonia and received IV Ig. He developed right hemiparesis 60 hours after the infusion. Blood investigations, echocardiography and carotid Doppler did not suggest vasculitis, thrombophilia or extracranial dissection. Brain computerised tomography (CT) showed acute left frontal and parietal infarcts. Initial magnetic resonance angiography (MRA) of cerebral vessels showed short segment attenuations of both proximal middle cerebral arteries and reduction in the calibre of bilateral supraclinoid internal carotid arteries. The boy was treated with enoxaparin and aspirin. He only had partial recovery of the hemiparesis on follow-up. A repeat MRA 13 months later showed parenchymal collateral vessels suggestive of moyamoya disease. We recommend imaging the cerebral vessels in children with a high risk of moyamoya before giving IV Ig.
We report a newborn infant girl, born to consanguineous parents, with recurrent intracranial hemorrhage secondary to congenital factor V deficiency with factor V inhibitor. Repeated transfusions of fresh-frozen plasma (FFP) and platelet concentrates, administrations of immunosuppressive therapy (prednisolone and cyclophosphamide), and intravenous immunoglobulin failed to normalize the coagulation profiles. Exchange transfusion followed-up by administrations of activated prothrombin complex and transfusions of FFP and platelet concentrates caused a temporary normalization of coagulation profile, enabling an insertion of ventriculoperitoneal (VP) shunt for progressive hydrocephalus. The treatment was complicated by thrombosis of left brachial artery and ischemia of left middle finger. The child finally died from another episode of intracranial hemorrhage 10 days after insertion of the VP shunt.
Matched MeSH terms: Immunoglobulins, Intravenous/therapeutic use
Neonatal alloimmune thrombocytopaenia (NAIT) generally results from platelet opsonisation by maternal antibodies against fetal platelet antigens inherited from the infant's father. Newborn monochorionic twins presented with petechial haemorrhages at 10 hours of life, along with severe thrombocytopaenia. Despite the initial treatment with platelet transfusions and intravenous immunoglobulin, they both had persistent thrombocytopaenia during their first 45 days of life. Class I human leucocyte antigen (HLA) antibodies with broad specificity against several HLA-B antigens were detected in the maternal serum. Weak antibodies against HLA-B57 and HLA-B58 in sera from both twins supported NAIT as the most likely diagnosis. Platelet transfusion requirements of the twins lasted for 7 weeks. Transfusion of HLA-matched platelet concentrates was more efficacious to manage thrombocytopaenia compared with platelet concentrates from random donors. Platelet genotyping and determination of HLA antibody specificity are needed to select compatible platelet units to expedite safe recovery from thrombocytopaenia in NAIT.