Atypical teratoid rhabdoid tumor in the cavernous sinus of a toddler presenting with oculomotor nerve palsy

Introduction

Atypical teratoid rhabdoid tumor (ATRT) is a rare, highly malignant, and aggressive tumor of infancy. Although the prognosis of ATRT has been extremely poor, recently, the first prospective study for ATRT demonstrated improvement of prognosis. On the other hands, oculomotor nerve palsy is rare in children and the most frequent etiology is congenital. To our knowledge, only a few ATRT cases presenting with oculomotor nerve palsy have been reported, but ATRT originating from the cavernous sinus (CS) has not yet been reported.

Case report

An 18-month-old girl with right oculomotor nerve palsy was admitted, and a small mass in the right CS was detected with brain MRI. Although she received steroid pulse therapy and antimicrobial therapy, the mass continued to enlarge. One month after admission, the mass was partially resected and diagnosed as ATRT. Multimodal therapy including anthracycline-based chemotherapy, intrathecal therapy, and cranial irradiation was performed. Twenty-nine months after resection, she was alive without tumor relapse, but the oculomotor nerve palsy persisted.

Conclusion

This is the first reported case of ATRT located in the CS presenting with oculomotor nerve palsy. This case was successfully treated with partial removal of the tumor, a new chemotherapy regimen for ATRT and cranial X-ray irradiation.     

Preoperative Sleep Complaints Are Associated with Poor Physical Recovery in the Months Following Cardiac Surgery

Background

Sleep disturbance is associated with poorer outcomes in cardiac patients, but little is known about the independent role of sleep quality in coronary artery bypass graft (CABG) patients.

Purpose

This study aims to examine the relationship between preoperative sleep complaints and post-operative emotional and physical recovery in CABG surgery patients, independently of demographic, clinical and mood factors.

Methods

Two hundred thirty CABG patients (aged 67.81?±?9.07 years) completed measures of self-reported sleep complaints before surgery and health-related quality of life (HRQoL), physical symptoms and pain 2 months after surgery.

Results

Greater sleep complaints prior to surgery were associated with greater physical symptoms, poorer physical HRQoL and greater sensory pain after surgery (p?<?0.05), but not with affective pain or mental HRQoL. Preoperative mood was not able to explain these associations.

Conclusions

Sleep complaints may be implicated in physical recovery from CABG surgery but further work is needed to understand the role of causal pathways.     

Bilateral radial nerve compression neuropathy in the newborn

Purpose

This study aims to discuss the diagnosis and management of radial nerve compression neuropathy in the newborn.

Methods

A personal case is presented, followed by a review and analysis of clinically similar cases identified via a PubMed search of published medical literature.

Results

We report a case of a term newborn with bilateral radial neuropathy at the humerus level. Despite severe axonal involvement in the electrophysiological evaluation, the patient showed complete bilateral recovery after 3 months of follow-up.

Conclusions

Isolated radial nerve palsy is a rare event in the newborn. The condition does not require surgical treatment and usually proceeds to full and rapid spontaneous recovery.     

Oberlin transfer and partial radial to axillary nerve neurotization to repair an explosive traumatic injury to the Brachial Plexus in a child: Case report

Purpose

Explosive injuries to the pediatric brachial plexus are exceedingly rare and as such are poorly characterized in the medical literature.

Methods

Herein, we describe an 8-year-old who was struck in the neck by a piece of shrapnel and suffered multiple vascular injuries in addition to a suspected avulsion of the cervical 5 and 6 ventral rami. The patient had a complete upper brachial plexus palsy and failed to demonstrate any clinical improvement at 6-months follow-up. He was taken to the operating from for a partial ulnar to musculocutaneous nerve neurotization as well as a partial radial to axillary nerve neurotization.

Results

The patient’s motor exam improved from a Medical Research Council scale 1 to 4+ for biceps brachii and 0 to 4 deltoid function with greater than 90° of shoulder abduction.

Conclusions

This outcome supports complex neurotization techniques as viable treatment options for persistent motor deficits following an upper brachial plexus injury in older, non-infant age, children.     

Ultra-Early Aneurysmal Rebleeding and Brainstem Destruction

Background

Early secondary neurologic deterioration after aneurysmal subarachnoid hemorrhage (SAH) may have many causes including rebleeding, hydrocephalus, parenchymal hematoma, or seizures.

Methods

Case report.

Results

A 69-year-old woman presented with thunderclap headache and nausea. A head computed tomography (CT) showed SAH. On initial evaluation she was awake, alert, and confused without focal neurologic deficits. Two episodes of marked clinical deterioration occurred, manifesting as acute unresponsiveness and fixed anisocoria. Serial head CTs showed massive extension of hemorrhage into the brainstem parenchyma and ventricles.

Conclusions

Sudden clinical deterioration after SAH with coma and a fixed ??blown?? pupil may result from hemorrhage extension into the brainstem parenchyma rather than oculomotor nerve injury from compression or stretch.     

Oberlin’s procedure in children with obstetric brachial plexus palsy

Purpose

Most cases of obstetric brachial plexus palsy (OBPP) involve C5 and C6 nerve roots (Erb’s palsy). In those cases, re-establishing the elbow flexion is the primary goal of surgery. The partial transfer of the ulnar nerve to the musculocutaneous nerve (Oberlin’s procedure) is widely used in adults, but incipient in children. The purpose of this study is to describe the results obtained with such procedure as regards the improvement of the elbow flexion and donor nerve morbidity.

Method

Thirteen children aged 9 to 15 months underwent Oberlin’s procedure. They were assessed preoperatively and 1 year postoperatively using the active movement scale and also according to the functionality of the affected limb. All of them were evaluated because of the possibility of movement loss resulting from the donor nerve. We used the non-parametric, statistic Wilcoxon signed rank test (α = 0,05) method.

Results

There was a significant improvement in the active elbow flexion between pre- and postoperative periods. Eleven children presented functional improvement. All of them maintained negative cookie test 1 year after the surgery. We did not observe any loss related to the donor nerve in terms of wrist flexion.

Conclusion

The results suggest that Oberlin’s procedure can be an effective and safe alternative to treat elbow flexion in Erb’s palsy.

     

Isolated pupil-sparing third-nerve palsy as the presenting sign of multiple sclerosis

A 27-year-old man presented with an isolated, pupil-sparing, partial third-nerve palsy. Etiologic investigations proved unrevealing. Subsequent development of an optic neuropathy prompted magnetic resonance imaging, which confirmed the clinical suspicion of multiple sclerosis. Rarely does multiple sclerosis present with oculomotor nerve involvement.     

Palliative epilepsy surgery in Aicardi syndrome: a case series and review of literature

Purpose

Aicardi syndrome (AS) is a severe neurodevelopmental disorder characterized by the triad of seizures, agenesis of corpus callosum, and chorioretinal lacunae. Seizures in AS are typically frequent, of various types, and refractory to medical therapy. Optimal treatment of seizures in AS remains undetermined.

Methods

We report a series of four patients with Aicardi syndrome who underwent surgical management of their epilepsy including two with corpus callosotomy (CC) of a partial corpus callosum and three with vagus nerve stimulator implantation.

Results

Seizure outcome was variable and ranged from near complete resolution of seizures to worsening of seizure profile. The most favorable seizure outcome was seen in a patient with partial agenesis of the corpus callosum treated with CC.

Conclusions

Seizure outcome following CC or vagus nerve stimulation in patients with Aicardi syndrome is variable. Although palliative epilepsy surgery may result in improvement in the seizure profile in some patients, studies on larger patient cohorts are needed to identify the precise role that surgery may play in the multidisciplinary approach to controlling seizures in Aicardi syndrome.     

Total ipsilateral C7 root neurotization to the upper trunk for isolated C5-C6 avulsion in obstetrical brachial plexus palsy: a preliminary technical report

Background

C5-C6 root avulsion in obstetrical brachial plexus palsy (OBPP) is a rare injury with poor prognosis usually associated with breech delivery. The treatment is challenging and requires high microsurgical skills. The triple nerve transfer (spinal accessory nerve, ulnar fascicles, and triceps long or lateral head branch) represents the gold standard treatment. The total ipsilateral C7 nerve root neurotization is a promising technique, which has never been described in OBPP.

Objective

The total ipsilateral C7 nerve root is used as a neurotizer transferred to the upper trunk as an alternative method to other intra- or extra-plexual reconstruction techniques.

Methods

During brachial plexus surgical exploration, an intraoperative neurostimulation was performed to confirm the integrity of C7 and the lesion of C5 and C6. The entire C7 nerve root and the upper trunk are cut. The C7 root was transferred to the upper trunk with a fibrin sealant.

Result

This technique was easily performed with a single approach and avoided intercalated nerve grafts. The C7 nerve root provided a large number of nerve fibers with an adequate diameter to be transferred to the upper trunk. We illustrated this technique with a typical case of a child at 8 years of follow-up.

Conclusion

The total ipsilateral transfer of the C7 root to the upper trunk is a viable alternative procedure for newborns with C5-C6 avulsion.     

Surgical outcome of oculomotor nerve palsy in pituitary adenoma

Oculomotor nerve palsy is a relatively rare symptom in pituitary adenoma compared to visual compromise or endocrine deficiency. The causes and recovery remain unclear. A total of 23 patients with pituitary adenomas presenting with oculomotor nerve palsy were reviewed. Patients were treated immediately with glucocorticoid therapy. Elective pure-endoscopic transsphenoidal surgery was used for decompression and histopathological confirmation. The clinical differences of patients with apoplectic (hemorrhage or infarction) (20 patients) and non-apoplectic tumors (three patients) were compared. In the apoplectic group, hemorrhage was noted in 13 patients and infarction in seven. Most patients presented with ptosis, followed by limited gaze and diplopia. In the long-term follow-up, the overall complete recovery rate was 19/23 (82.6%): 18/20 in the apoplectic group (90%), and one in three patients in the non-apoplectic group (33%). The median recovery time was 9 days after surgical decompression; and early treatment resulted in early recovery (p = 0.03). Patients with pupil-sparing pituitary adenoma recovered more rapidly than those with pupil involvement (p = 0.012). Patients with minor symptoms recovered earlier than patients with complete palsy (p = 0.003). MRI revealed that the tumor had invaded the interclinoid ligament region in all patients. We conclude that oculomotor nerve palsy usually occurs in patients with apoplectic adenomas, especially those with hemorrhage. Early treatment, pupil-sparing, and minor oculomotor symptoms are factors indicating a good recovery. Endoscopic transsphenoidal surgical decompression achieved good results in this study.     

Johan Georg Ræder (1889–1959) and paratrigeminal sympathetic paresis

Introduction

Johan Georg Ræder (1889–1959) was the most eminent Norwegian ophthalmologist in the early decades of the last century. Ræder made significant contributions to our current understanding of glaucoma. He is remembered for a syndrome he described, that of trigeminal nerve neuralgia and/or paresis and incomplete Horner’s syndrome (oculopupillary sympathetic paresis).

Discussion

Here, Ræder's biography, scientific contributions, and a thorough review of his original report on paratrigeminal sympathetic paresis are presented. Ræder's syndrome may reflect a lesion of the middle cranial fossa, which involves oculopupillary sympathetic fibers that originate from the internal carotid artery plexus and travel with the trigeminal and oculomotor nerves.     

Reverse Locked-In Syndrome

Background

Basilar artery occlusion can cause locked-in syndrome, which is characterized by quadriplegia, anarthria, and limited communication via eye movements. Here, we describe an uncommon stroke syndrome associated with endovascular recanalization of the top of the basilar artery: “reverse locked-in syndrome.”

Methods

We report the case of a patient with atypical neurological deficits caused by acute ischemic stroke of the midbrain tegmentum. We perform neuroanatomic localization of the patient’s infarcts by mapping the magnetic resonance imaging (MRI) data onto a brainstem atlas.

Results

A 61-year-old man presented with acute coma and quadriplegia due to top of the basilar artery occlusion. He underwent emergent endovascular thrombectomy, with successful recanalization of the basilar artery at 4 h and 43 min post-ictus. The patient regained consciousness and purposeful movement in all four extremities, but the post-procedure neurological examination demonstrated bilateral ptosis with complete pupillary and oculomotor paralysis. MRI revealed infarction of the bilateral oculomotor nuclei in the midbrain tegmentum. At 9-month follow-up, he had anisocoria and dysconjugate gaze, but was living at home and required minimal assistance in performing all activities of daily living.

Conclusions

Since the patient’s deficits were the exact opposite of those described in locked-in syndrome, we propose the term “reverse locked-in syndrome” to describe this neurological entity characterized by bilateral ptosis, non-reactive pupils, and ophthalmoplegia with preservation of consciousness and extremity motor function.

     

A patient with reversible pupil-sparing Weber's syndrome

This is a case report of a lady who presented with pupil-sparing Weber's syndrome. She had left oculomotor nerve palsy with normal pupil and right hemiparesis. The patient subsequently made a good recovery. An ischemic lesion of the lower mid-brain was demonstrated on the MRI scan of the brain, which corresponds to the motor nucleus of the oculomotor nerve. The article also describes the neuroanatomy of the oculomotor nerve and how its partial lesions lead to sparing of the pupil. In addition, this case report documents that a pupil-sparing Weber's syndrome could be reversible.     

Efficacy and Feasibility of a Tele-health Intervention for Acute Coronary Syndrome Patients with Depression: Results of the “MoodCare” Randomized Controlled Trial

Background

Depression is common after a cardiac event, yet there remain few approaches to management that are both effective and scalable.

Purpose

We aimed to evaluate the 6-month efficacy and feasibility of a tele-health program (MoodCare) that integrates depression management into a cardiovascular disease risk reduction program for acute coronary syndrome patients with low mood.

Methods

A two-arm, parallel, randomized design was used comprising 121 patients admitted to one of six hospitals for acute coronary syndrome.

Results

Significant treatment effects were observed for Patient Health Questionnaire 9 (PHQ9) depression (mean difference [change]?=??1.8; p?=?0.025; effect size: d?=?0.36) for the overall sample, when compared with usual medical care. Results were more pronounced effects for those with a history of depression (mean difference [change]?=??2.7; p?=?0.043; effect size: d?=?0.65).

Conclusions

MoodCare was effective for improving depression in acute coronary syndrome patients, producing effect sizes exceeding those of some face-to-face psychotherapeutic interventions and pharmacotherapy. (Trial Registration Number: ACTRN1260900038623.)     

Diffusion tensor imaging of the superior cerebellar peduncle identifies patients with posterior fossa syndrome

Introduction

Posterior fossa tumors are the most common brain tumor of children. Aggressive resection correlates with long-term survival. A high incidence of posterior fossa syndrome (PFS), impairing the quality of life in many survivors, has been attributed to damage to bilateral dentate nucleus or to cerebellar output pathways. Using diffusion tensor imaging (DTI), we examined the involvement of the dentothalamic tracts, specifically the superior cerebellar peduncle (SCP), in patients with posterior fossa tumors and the association with PFS.

Methods

DTI studies were performed postoperatively in patients with midline (n?=?12), lateral cerebellar tumors (n?=?4), and controls. The location and visibility of the SCP were determined. The postoperative course was recorded, especially with regard to PFS, cranial nerve deficits, and oculomotor function.

Results

The SCP travels immediately adjacent to the lateral wall of the fourth ventricle and just medial to the middle cerebellar peduncle. Patients with midline tumors that still had observable SCP did not develop posterior fossa syndrome (N?=?7). SCPs were absent, on either preoperative (N?=?1, no postoperative study available) or postoperative studies (N?=?4), in the five patients who developed PFS. Oculomotor deficits of tracking were observed in patients independent of PFS or SCP involvement.

Conclusion

PFS can occur with bilateral injury to the outflow from dentate nuclei. In children with PFS, this may occur due to bilateral injury to the superior cerebellar peduncle. These tracts sit immediately adjacent to the wall of the ventricle and are highly vulnerable when an aggressive resection for these tumors is performed.     

Attachment anxiety predicts IL-6 and length of hospital stay in coronary artery bypass graft surgery (CABG) patients

Objective

The mechanisms underlying the association between adult attachment and health are not well understood. In the current study, we investigated the relationship between attachment anxiety, attachment avoidance, inflammation, and length of hospital stay in coronary artery bypass graft (CABG) surgery patients.

Method

167 CABG patients completed an attachment questionnaire prior to surgery, and blood samples were taken before and after surgery to assess inflammatory activity.

Results

We found that attachment anxiety predicted higher plasma interleukin 6 (IL-6) concentration, and this association was mediated by self-reported sleep quality. Anxious attachment also predicted longer hospital stays following CABG surgery, even after controlling for demographic and clinical factors.

Conclusion

These data suggest that increased levels of IL-6 may be a process linking adult attachment anxiety with health outcomes.     

Combining carotid endarterectomy with off-pump coronary artery bypass graft surgery is safe and effective

Background:

We, as neurologists, are frequently consulted to give neurological clearance for surgery in patients who are undergoing coronary artery bypass graft (CABG) surgery and have suffered from stroke or transient ischemic attack (TIA) in past. Similarly clearance is also sought in another group of patients who, though have not suffered from stroke or TIA, but found to have significant carotid stenosis on routine screening prior to surgery. Cardiac surgeons and anesthetists want to know the risk of perioperative stroke in such patients and should carotid endarterectomy (CEA) be done along with CABG. In absence of any clear-cut guideline, neurologists often fail to give any specific recommendation.

Aim:

To find out safety and efficacy of synchronous CEA in patients undergoing CABG.

Design:

Retrospective study.

Materials and Methods:

Out of 3,700 patients who underwent CABG, 150 were found to have severe carotid stenosis of >70%. Out of this, 46 patients with >80% stenosis (three symptomatic and 43 asymptomatic) and one patient with >70% symptomatic carotid stenosis (TIA within last 2 weeks) were taken for simultaneous CEA along with CABG. These three symptomatic carotid patients had suffered from stroke within last 6 months.

Results:

One patient with asymptomatic near total occlusion of carotid artery suffered from hyperperfusion syndrome. None suffered from ischemic stroke, myocardial infarction (MI), or death during perioperative period.

Conclusion:

Combining CEA along with CABG is a safe and effective procedure.     

Bedside Lung Ultrasound: A Case of Neurogenic Pulmonary Edema

Background

Neurogenic pulmonary edema (NPE) is an acute life-threatening complication associated with many forms of central nervous system injury. NPE usually appears within minutes to hours after injury and has a high mortality rate if not recognized and treated appropriately. Lung ultrasound quickly provides at the bedside relevant information on the state of aeration and ventilation of the lung.

Methods

We describe a case report of acute respiratory insufficiency after posterior cranial fossa surgery.

Results

The patient underwent a subtotal meningiomectomy. Postoperative course was complicated by respiratory failure with unstable hemodynamic parameters. The pulmonary edema was suspected, and sonography examination was performed. Lung ultrasound showed typical signs for non-cardiogenic pulmonary edema. Transthoracic echocardiography showed preserved left ventricle systolic function, but signs of the severe hypovolemia were found. We corrected for the preload and ventilator support settings. Within 24 h, her respiratory status improved with a resolution of the pulmonary edema.

Conclusions

Lung ultrasound at the bedside can provide accurate information on lung status in neurocritically ill patients with acute respiratory failure. The addition of transthoracic echocardiography to lung sonography provides an additive insight on the eventual pulmonary involvement. Lung ultrasound has the potential to become a reference tool for bedside dynamic respiratory monitoring in the Neuro ICU.     

Clinical and nerve conduction features in Guillain−Barré syndrome associated with Zika virus infection in Cúcuta,Colombia

Background and purpose

Zika virus (ZIKV) infection has been associated with an increased incidence of Guillain?Barré syndrome (GBS) but the relative frequency of acute inflammatory demyelinating polyradiculoneuropathy (AIDP) and axonal GBS subtypes is controversial.

Methods

Twenty GBS patients diagnosed according to the Brighton criteria during the ZIKV outbreak in Cúcuta, Colombia, were evaluated clinically and electrophysiologically. The electrodiagnosis of GBS subtypes was made according to a recently described criteria set that demonstrated a high diagnostic accuracy on the basis of a single test. The electrophysiological features of 34 Italian AIDP patients were used as control.

Results

All patients had symptoms compatible with ZIKV infection before the onset of GBS and ZIKV infection was laboratory confirmed through a plaque reduction neutralization test (PRNT₉₀) in 100% of patients. The median time from onset of ZIKV infection symptoms to GBS was 5 days (interquartile range 1–6 days). Cranial nerve palsy was present in 85% of patients (facial palsy in 75%, bulbar nerve involvement in 60%), autonomic dysfunction in 85%, and 50% of patients required invasive mechanical ventilation. AIDP was diagnosed in 70% of patients. 40% of nerves of AIDP patients showed a prevalent distal demyelinating involvement but this pattern was not different from the Italian AIDP patients without ZIKV infection.

Conclusions

Guillain?Barré syndrome associated with ZIKV infection in Cúcuta is characterized by a high frequency of cranial nerve involvement, autonomic dysfunction and requirement of mechanical ventilation indicating an aggressive and severe course. AIDP is the most frequent electrophysiological subtype. Demyelination is prevalent distally but this pattern is not specific for ZIKV infection.

     

Pupil-sparing third nerve palsies and hemiataxia: Claude’s and reverse Claude’s syndrome

We report two patients with midbrain infarction with pupil-sparing third nerve palsies and hemiataxia: one with contralateral ataxia (Claude’s syndrome) and one with ipsilateral ataxia (which we refer to as reverse Claude’s syndrome). We highlight the importance of a thorough neurologic evaluation with partial oculomotor palsies and describe, to our knowledge, the fourth account in the literature of a pupil-sparing third nerve palsy with ipsilateral cerebellar ataxia.