Naresh Mullaguri

Pediatric familial neuromyelitis optica in two sisters with long term follow-up.

Neuromyelitis optica causes bilateral optic neuritis and longitudinal extensive transverse myelitis. Although usually sporadic, 3% of cases of neuromyelitis optica are familial. The interval over which attacks continue and the long term prognosis for pediatric-onset neuromyelitis optica are not well defined. We describe two patients with pediatric familial neuromyelitis optica with the longest clinical follow-up of a pediatric case reported in the literature to our knowledge. One woman developed blindness with bilateral eye involvement within a few weeks at age 3. This was followed by transverse myelitis with paraparesis at age 19 leading to diagnosis of neuromyelitis optica. Her serum anti-aquaporin 4 antibody was later found to be positive. She continued with sporadic myelitis-related relapses but remained ambulant until age 40 when she had a more severe relapse. There was evidence of longitudinal extensive T2 hyperintensity in the thoracic spinal cord. Her sister also developed blindness at age 3.5 followed by myelitis 1year later with multiple relapses of gait impairment until her death from pneumonia at age 21. These patients represent the rare occurrence of neuromyelitis optica in children within the same family and show that this disease can have prolonged periods of remission but a continued tendency to relapse, supporting the need for lifelong immunosuppression.

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

BackgroundWernekink 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 presentationA 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.ConclusionsWernekink 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.

Trans-cranial Doppler as an Ancillary Study Supporting Irreversible Brain Injury in a Post Cardiac Arrest Patient on Extracorporeal Membrane Oxygenation

Obtaining neuroimaging in patients on cardiopulmonary support devices such as extracorporeal membrane oxygenation (ECMO) can be challenging, given the complexities in monitoring, instrumentation, and associated hemodynamic lability. Transcranial Doppler (TCD) is used as an ancillary test for the assessment of cerebral circulatory arrest, but its use in non-pulsatile blood flow in venoarterial (VA) ECMO is not well described. We report the use of TCD in a patient on VA ECMO post-cardiac arrest for evaluation of death by neurological criteria. A 72-year-old female was admitted for elective trans-catheter aortic valve replacement. Her postoperative course was complicated by hemo-pericardium evolving into pulseless electrical activity causing cardiac arrest. She was resuscitated with return of spontaneous circulation and initiated on VA ECMO and intra-aortic balloon pump for cardiogenic shock. Over the next few days, serial evaluations persistently showed a poor neurological examination. She was too unstable to transport for neuroimaging. Evaluation for death by neurological criteria was performed with a clinical examination, apnea testing, and TCD as an ancillary study. TCD showed systolic spikes supporting an impression of cerebral circulatory arrest consistent with an irreversible brain injury.

Intracranial Calcification Masquerading as Hemorrhage in a Patient with Multiple Myeloma Presenting with Facial Neuropathy

Multiple myeloma is an immunoglobulin-producing plasma cell neoplasm that commonly affects the bones, kidneys, the hematopoietic system, and rarely the nervous system. Peripheral nervous system involvement in the form of cranial neuropathy, radiculopathy, and polyneuropathy are common. Compressive myelopathy constitutes the majority of central nervous system disorders followed by cerebrovascular disorders, intracranial plasmacytomas, and leptomeningeal myelomatosis. Cerebrovascular complications such as acute ischemic stroke and transient ischemic attack are not uncommon. Intracerebral hemorrhage, although infrequent, can be secondary to refractory hypertension from renal failure and intratumoral hemorrhage in intracerebral plasmacytomas. Metastatic calcifications in lungs, liver, and skin with high levels of serum calcium and phosphate are seen in patients with multiple myeloma, but intracerebral calcifications are not common. We report an unusual case of intracranial calcification masquerading as acute intracerebral hemorrhage in a patient presenting with acute facial weakness.

Cocaine Induced Bilateral Posterior Inferior Cerebellar Artery and Hippocampal Infarction

Cocaine is one of the most commonly abused recreational drugs, second only to marijuana. It blocks the reuptake of neurotransmitters such as norepinephrine and dopamine, that leads to persistent post-synaptic stimulation responsible for its excitatory effects. Cocaine-related strokes, both ischemic and hemorrhagic, have been well described in the literature and cerebral vasospasm is hypothesized as one of the major mechanisms responsible for the presentation. Although cases of posterior circulation infarction were previously reported, we herein report a rare presentation of a cocaine-induced bilateral posterior inferior cerebellar artery and hippocampal infarction in a middle-aged female.

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.

Brugada syndrome in a patient with amyotrophic lateral sclerosis: a case report

BackgroundAmyotrophic lateral sclerosis is a fatal neuromuscular disorder characterized by progressive death of the upper and lower motor neurons in the central nervous system. Patients with this disease die mostly as a result of respiratory failure; however, owing to prolonged survival through assisted ventilation, cardiovascular causes are increasingly responsible for mortality. We report what is to the best of our knowledge the first case of type 2 Brugada syndrome causing ventricular tachyarrhythmia and cardiac arrest in a patient with upper limb onset amyotrophic lateral sclerosis.Case presentationA 48-year-old Caucasian woman with a significant past medical history of papillary thyroid carcinoma status postresection, pulmonary embolism on anticoagulation, and a recent diagnosis of right upper limb-onset amyotrophic lateral sclerosis presented to the emergency department of our hospital with acute on chronic shortness of breath. On further evaluation, she was found to have hypoxic and hypercapnic respiratory failure and was placed on bilevel positive airway pressure ventilation. Her 12-lead electrocardiogram showed sinus rhythm with J-point elevation, saddle-shaped ST segment elevation, predominantly in V1 and V2 with no significant QTc prolongation. No troponin elevation was noted in her laboratory workup. Because she was unable to protect her airway, a decision was made to intubate her. After 1 minute of induction with etomidate and succinylcholine, she went into pulseless ventricular tachycardia and fibrillation requiring three cycles of cardiopulmonary resuscitation with high-quality chest compressions, three doses of epinephrine, and a loading dose of amiodarone prior to return of spontaneous circulation. She was further evaluated by cardiology services and was diagnosed with type 2 Brugada syndrome, for which she was started on quinidine. Her respiratory failure and the drugs she received for intubation likely caused her ventricular tachycardia to occur in conjunction with an underlying Brugada pattern seen on an electrocardiogram. The results of evaluation of her genetic panel for Brugada syndrome were negative. She was subsequently discharged to home in stable condition after a 10-day hospital stay.ConclusionsAmyotrophic lateral sclerosis is a progressive neuromuscular disorder with significant mortality. Respiratory failure is the leading cause of death, but lately, owing to increased survival associated with early tracheostomy and positive pressure ventilation, there has been an increasing trend in the identification of cardiovascular causes of mortality, especially arrhythmias, that may need periodic electrocardiographic surveillance.

Pitfall in Pupillometry: Exaggerated Ciliospinal Reflex in a Patient in Barbiturate Coma Mimicking a Nonreactive Pupil

Although a neurological examination is fundamental to the evaluation of comatose patients, it is less reliable in a medically induced coma. A commonly misinterpreted finding in patients in a pentobarbital coma is altered pupillary reactivity secondary to an exaggerated ciliospinal reflex. Recognizing an exaggerated ciliospinal reflex in patients in a pentobarbital coma is important and may prevent unnecessary intervention. We present a patient induced in a pentobarbital coma for the treatment of status epilepticus who exhibited a nonreactive pupil secondary to an exaggerated ciliospinal reflex confirmed by pupillometry. We also discuss the anatomy of the ciliospinal reflex and literature regarding its clinical relevance.

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 Graves disease need to be evaluated. Case Report A 69-year-old female with seropositive myasthenia gravis (MG), Graves 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 Graves disease. These patients might benefit from meticulous fluid status and cardiac monitoring while administering rescue treatments like IVIG and PLEX.