Wernekink commissure syndrome with palatal myoclonus at onset: a case report and review of the literature

Abstract Background Wernekink commissure syndrome causes a peculiar combination of internuclear ophthalmoplegia, dysarthria, and delayed-onset palatal myoclonus. Palatal myoclonus is thought to be secondary to delayed hypertrophic degeneration of the bilateral inferior olivary nuclei secondary to involvement of bilateral dentatoolivary tract. We describe a case of a patient with early-onset palatal myoclonus. Case presentation A 53-year-old Caucasian man with several vascular risk factors presented to our emergency room with slurred speech, double vision, difficulty with swallowing and walking, and rhythmic contractions of the soft palate. Brain magnetic resonance imaging showed an acute infarct of the right caudal midbrain and an old infarct of the right medulla. We hypothesize that the cause of early palatal myoclonus in our patient was a two-hit mechanism with degeneration of the right olivary nucleus resulting from prior right medullary lacunar stroke with the new infarct affecting the dentato-rubro-olivary tract on the left side, causing bilateral dysfunction initiating palatal myoclonus. Conclusions Wernekink commissure syndrome with palatal myoclonus at onset suggests the presence of a prior ischemic insult in the medulla. Careful examination is important to identification of this presentation

Decompression Hemicraniectomy for Refractory Intracranial Hypertension in Reversible Cerebral Vasoconstriction Syndrome

Reversible cerebral vasoconstriction syndrome (RCVS) is a disorder of dysregulation of cerebrovascular tone resulting in transient segmental vasoconstriction which resolves in 1–3 months. Cerebral edema is an underrecognized complication in RCVS. It is likely multifactorial. This edema can lead to intracranial hypertension that can be refractory to medical management. Limited evidence exists regarding surgical management of intracranial hypertension in RCVS. We present a 29-year-old Caucasian right-handed female patient with a medical history of migraine, polysubstance abuse presented to the emergency department (ED) daily for 3 days with the chief complaint of recurrent thunderclap headache. She declined neuroimaging and lumbar puncture. She was treated for migraine with abortive medications with no improvement. During the third ED visit, she became lethargic with right-sided homonymous hemianopia. Computerized tomography of the brain showed left parietal intracerebral hemorrhage with intraventricular extension, cortical subarachnoid hemorrhage, and diffuse cerebral edema. Digital subtraction angiography showed multifocal moderate-to-severe segmental vasoconstriction suggestive of vasculopathy. Oral verapamil was initiated. Continuous intracranial pressure monitoring showed uncontrolled intracranial hypertension, despite maximal medical management with hyperosmolar therapy, induced coma, and hypothermia. Decompressive hemicraniectomy with duraplasty was performed for refractory intracranial hypertension. We provisionally diagnosed her with RCVS. She was discharged to inpatient rehabilitation with residual right homonymous hemianopia. Transcranial Doppler study during follow-up showed improved mean flow velocities. She continued to have residual cognitive deficits with complete resolution of headache

False-Negative Platelet Factor 4 Antibodies and Serotonin Release Assay and the Utility of Repeat Testing in the Diagnosis of Heparin-Induced Thrombocytopenia and Thrombosis

Objective. To report a case of false-negative serological tests in the diagnosis of heparin-induced thrombocytopenia (HIT) followed by a brief review of the literature on this topic. Case Presentation. A 75-year-old Caucasian female patient was admitted with a traumatic right ankle fracture that required open reduction and internal fixation. Despite postoperative subcutaneous heparin chemoprophylaxis, she developed deep vein thrombosis (DVT) and pulmonary embolism (PE) on day 4 and subsequently started on continuous heparin infusion. On day 5, she suffered a stroke from a complete occlusion of the right common carotid artery with tandem occlusion of the right middle cerebral artery. She underwent successful thrombectomy of both arteries. The proposed stroke mechanism was paradoxical embolism through a patent foramen ovale. Over the next few days, thrombocytopenia was noted, the heparin drip was stopped, and HIT antibodies (antibodies targeting the complex of platelet factor 4 and heparin; PF4-H AB) and serotonin release assay (SRA) tests were sent. Because of the suspicion for HIT, she was started on bivalirudin with subsequent improvement in platelet count. Initial PF4-H AB and SRA tests were negative, bivalirudin was stopped, and heparin was restarted. Subsequently, her platelets trended down, again raising clinical suspicion of HIT. Repeat PF4-H AB and SRA testing resulted positive. Conclusions. A positive SRA in the appropriate context is considered for the diagnosis of heparin-induced thrombocytopenia. This case report highlights that false-negative serological evaluation is possible early in the course of the disease. Repeat testing is recommended in patients with high clinical suspicion

White Matter Changes in Corpus Callosum in a Patient with Idiopathic Normal Pressure Hydrocephalus

Idiopathic normal pressure hydrocephalus (INPH) is characterized by the clinical triad of gait and cognitive dysfunction and urinary incontinence. Ventriculoperitoneal (VP) shunting is often required for treatment. Review of literature shows few case reports discussing benign magnetic resonance imaging (MRI) T2 hyperintense changes in the corpus callosum of NPH patients after shunting due to mechanical compression of the middle and posterior regions of the body against falx cerebri leading to ischemic demyelination. These changes can be a delayed phenomenon and may interfere with clinical evaluation and may lead to unnecessary procedures and investigations. We present a patient with NPH who was admitted to the neurocritical care unit in coma with quetiapine and trazodone overdose. Diffuse changes in the body of the corpus callosum were seen on MRI suspicious for acute vasogenic edema due to drug overdose. However, it was later determined to be due to the VP shunting for the NPH. We report this case to raise the awareness of neuroimaging changes in patients with NPH who have VP shunting

Dihydroergotamine Complicating Reversible Cerebral Vasoconstriction Syndrome in Status Migrainosus

Reversible cerebral vasoconstriction syndrome (RCVS) is a clinicoradiological syndrome that occurs due to dysfunction of cerebrovascular autoregulation. It is characterized by recurrent thunderclap headache from cerebral vasoconstriction which can cause ischemic infarction, spontaneous intraparenchymal and subarachnoid hemorrhage. This syndrome can be triggered by a variety of etiologies including medications, infectious, and inflammatory conditions. The diagnosis is often delayed due to unawareness among the health-care providers and delayed neuroimaging evidence of vasoconstriction with or without ischemic and/or hemorrhagic infarction. Status migrainosus is a prevalent condition requiring emergency room visits and inpatient admission. Thus, patients with RCVS can be easily misdiagnosed with migraine. We report a patient with RCVS misdiagnosed as status migrainosus with visual aura, treated with intravenous dihydroergotamine with worsening of cerebral vasoconstriction and lead to ischemic and hemorrhagic complications. We discuss this complication and provide guidance on differentiating between migraine and RCVS

A Case Report of Recurrent Takotsubo Cardiomyopathy in a Patient during Myasthenia Crisis

Introduction. Patients with myasthenia crisis can develop Takotsubo stress cardiomyopathy (SC) due to emotional or physical stress and high level of circulating catecholamines. We report a patient who developed recurrent Takotsubo cardiomyopathy during myasthenia crisis. Coexisting autoimmune disorders known to precipitate stress cardiomyopathy like Grave’s disease need to be evaluated. Case Report. A 69-year-old female with seropositive myasthenia gravis (MG), Grave’s disease, and coronary artery disease on monthly infusion of intravenous immunoglobulin (IVIG), prednisone, pyridostigmine, and methimazole presented with shortness of breath and chest pain. Electrocardiogram (ECG) showed ST elevation in anterolateral leads with troponemia. Coronary angiogram was unremarkable for occlusive coronary disease with left ventriculogram showing reduced wall motion with apical and mid left ventricle (LV) hypokinesis suggestive of Takotsubo stress cardiomyopathy. Her symptoms were attributed to MG crisis. Her symptoms, ECG, and echocardiographic findings resolved after five cycles of plasma exchange (PLEX). She had another similar episode one year later during myasthenia crisis with subsequent resolution in 10 days after PLEX. Conclusion. Takotsubo cardiomyopathy can be one of the manifestations of myasthenia crisis with or without coexisting Grave’s disease. These patients might benefit from meticulous fluid status and cardiac monitoring while administering rescue treatments like IVIG and PLEX

Super-Refractory Status Epilepticus Treated with High Dose Perampanel: Case Series and Review of the Literature

Introduction. Acute symptomatic seizures are frequent in the critically ill patient and can be difficult to treat. The novel anticonvulsant perampanel may be effective in the treatment of status epilepticus considering its mechanism of action of being an AMPA antagonist. We present four cases of super refractory status epilepticus treated with high dose perampanel. Method. Case report. Cases. Four patients were treated with perampanel for their refractory status epilepticus. One patient had new onset refractory status epilepticus of unknown etiology. Three other patients had status epilepticus as a result of their cardiac arrest. Two of the cardiac arrest patients had myoclonus. In all patients, the additional of perampanel resulted in a reduction of seizure burden without affecting hemodynamics or hepatic or renal function. Conclusion. Perampanel may be effective in the treatment of super-refractory status epilepticus of varying etiologies. A larger, prospective study is needed to further assess this therapy