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

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

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