Antagonizing dabigatran by idarucizumab in cases of ischemic stroke or intracranial hemorrhage in Germany-Updated series of 120 cases.

BACKGROUND: Idarucizumab is a monoclonal antibody fragment with high affinity for dabigatran reversing its anticoagulant effects within minutes. Thereby, patients with acute ischemic stroke who are on dabigatran treatment may become eligible for thrombolysis with recombinant tissue-type plasminogen activator (rt-PA). In patients on dabigatran with intracerebral hemorrhage idarucizumab could prevent lesion growth. AIMS: To provide insights into the clinical use of idarucizumab in patients under effective dabigatran anticoagulation presenting with signs of acute ischemic stroke or intracranial hemorrhage. METHODS: Retrospective data collected from German neurological/neurosurgical departments administering idarucizumab following product launch from January 2016 to August 2018 were used. RESULTS: One-hundred and twenty stroke patients received idarucizumab in 61 stroke centers. Eighty patients treated with dabigatran presented with ischemic stroke and 40 patients suffered intracranial bleeding (intracerebral hemorrhage (ICH) in n = 27). In patients receiving intravenous thrombolysis with rt-PA following idarucizumab, 78% showed a median improvement of 7 points in National Institutes of Health Stroke Scale. No bleeding complications were reported. Hematoma growth was observed in 3 out of 27 patients with ICH. Outcome was favorable with a median National Institutes of Health Stroke Scale improvement of 4 points and modified Rankin score 0-3 in 61%. Six out of 40 individuals (15%) with intracranial bleeding died during hospital stay. CONCLUSION: Administration of rt-PA after reversal of dabigatran activity with idarucizumab in case of acute ischemic stroke seems feasible, effective, and safe. In dabigatran-associated intracranial hemorrhage, idarucizumab appears to prevent hematoma growth and to improve outcome.

Miller Fisher syndrome with presynaptic neuromuscular transmission disorder.

Miller Fisher syndrome is defined by a triad of symptoms, namely areflexia, ataxia, and ophthalmoparesis. The ophthalmoparesis is mostly severe, undulating weakness of eye movements with ptosis and increased fatigability resembling a neuromuscular transmission disorder. We present a 52-year-old man with severe Miller Fisher syndrome with a high level of anti-GQ1b antibodies and a presynaptic type of neuromuscular transmission disorder. The diagnosis was confirmed by stimulated single-fiber electromyography with the use of a concentric needle electrode and various stimulation rates.

Ulnar nerve at the elbow - normative nerve conduction study.

INTRODUCTION: A goal of our work was to perform nerve conduction studies (NCSs) of the ulnar nerve focused on the nerve conduction across the elbow on a sufficiently large cohort of healthy subjects in order to generate reliable reference data. METHODS: We examined the ulnar nerve in a position with the elbow flexion of 90o from horizontal. Motor response was recorded from the abductor digiti minimi muscle (ADM) and the first dorsal interosseous muscle (FDI). RESULTS: In our sample of 227 healthy volunteers we have examined 380 upper arms with the following results: amplitude (Amp)-CMAP(wrist) for ADM 9.6 ± 2.3 mV, MNCV at the forearm 60.4 ± 5.2 m/s, MNCV across the elbow 57.1 ± 5.9 m/s. DISCUSSION: Our study showed that motor NCSs of the ulnar nerve above elbow (AE) and below elbow (BE) in a sufficiently large cohort using methodology recommended by AANEM gave results well comparable for registration from FDI and ADM.

Myasthenia gravis, Castleman disease, pemphigus, and anti-phospholipid syndrome.

INTRODUCTION: Myasthenia gravis is an autoimmune disease marked by neuromuscular transmission failure at the neuromuscular junction. Castleman disease is a rare lymphoproliferative disease characterized by non-cancerous angiofolicular hyperplasia of lymphatic tissue. METHODS AND RESULTS: We describe a young man with rapid, successive manifestations of myasthenia gravis, a solitary form of Castleman disease, pemphigus vulgaris, and anti-phospholipid syndrome, which resulted in 2 ischemic cerebrovascular events that caused a severe central neurological deficit. DISCUSSION: We were unable to find a similar case in the literature, but we hypothesize that the temporal concidence of these clinical entities may be related to a common immunological pathway, such as B-cell activation. Therefore, we treated the patient with an immunosuppressant and anticoagulant treatment, as well as rituximab, a monoclonal antibody therapy against CD20+.

Exposure to iodomethane and dichloromethane associated with a confusional state.

Dichloromethane and iodomethane are colorless relatively volatile liquids, which are used as solvents in chemical manufacturing processes. The major route of exposure is via inhalation and to a lesser extent through the skin and digestive tract. Both substances are characterized by significant neurotoxic effects. A 37-year-old chemist subjected to long-term inhalation exposure to both substances had been experiencing headaches, dizziness and fatigue for about 5 years. After an exceptional acute exposure, the man developed ataxia, increasing inhibition and a confusional and delirious state. Magnetic resonance imaging (MRI) of his brain in the acute state demonstrated the presence of a T2-hyperintense lesion in the splenium of the corpus callosum, suggestive as myelinolysis. On MRI 16 days later, the MRI changes had completely resolved and the clinical picture had improved significantly. To the best of our knowledge, this is the first published report of a case of "reversible focal splenial lesion syndrome of the corpus callosum", which was likely caused by industrial toxic substances.

Stiff-person syndrome following tick-borne meningoencephalitis.

Stiff-person syndrome (SPS) is a rare disorder characterized by muscle stiffness and painful spasms. Misdiagnosis may occur due to the fact that the clinical picture of SPS is often atypical. The main pathophysiologic mechanism underlying the development of SPS is insufficient inhibition at the cortical and spinal levels. There is good evidence for a primary autoimmune etiology. A 61-year-old man was admitted to a neurological department due to muscle hypertonia with episodic attacks of painful spasms predominantly affecting axial muscles. The symptoms developed shortly after tickborne meningoencephalitis. Electromyography (EMG) revealed signs of continuous motor unit activity. Antibodies against glutamate decarboxylase (anti-GAD) were highly elevated. We present a case of a man who developed clinically severe anti-GAD positive SPS, provoked by tick-borne encephalitis. After therapeutic plasma exchange (TPE) a rapid, temporary improvement of the clinical and neurophysiological findings was noted. Only after being placed on long-term immunosuppression did the patient achieve stable recovery. This case supports the importance of EMG findings and demonstrates the effect of TPE as well as the need for chronic immunosuppression in severe cases of SPS.