A complicated Chiari type 1 malformation and holocord syrinx as a likely cause for heel pain.

BACKGROUND: Chiari malformations are a rare group of rhomboencephalic abnormalities involving the brain, craniocervical junction and spine. They may manifest in a variety of clinical presentations which relate to the variable involvement of the cerebellum, brainstem, lower cranial nerves, spinal cord and altered CSF flow dynamics. METHOD: We report an unusual case of incidental diagnosis of a type I Chiari malformation with secondary cystic cerebellar tonsillar encephalomalacia and holocord syrinx following investigation of a 5YO girl presenting with heel swelling related to progressive neuropathic osteoarthropathy of the posterior calcaneal body and apophysis. RESULT: The child was treated with decompressive suboccipital craniectomy and C1 laminectomy and tonsillar resection. Cerebellar tonsillar gliosis and cystic degeneration were confirmed on histopathology. Referral for ongoing engagement with occupational and physical therapy. CONCLUSION: Most type I Chiari malformations in the paediatric population are incidental and asymptomatic. Neurological symptoms are typically mild and relate to altered CSF flow dynamics; however, we present a complex case of type I Chiari malformation with an unusual constellation of associated complications.

Middle meningeal artery embolization for chronic subdural hematoma in a young patient with refractory thrombocytopenia secondary to leukemia: more evidence for a paradigm shift? Illustrative case.

BACKGROUND: Chronic subdural hematoma (CSDH) is one of the most common neurosurgical presentations, with an increasing number now also presenting with concurrent thrombocytopenia. Although middle meningeal artery (MMA) embolization has been considered in elderly patients with high comorbidities, it may permit treatment of CSDH in patients who are at high risk for recurrence or deemed unsuitable for surgical management due to thrombocytopenia. OBSERVATIONS: A 35-year-old man who had severe thrombocytopenia due to blast cell crisis with chronic myeloid leukemia developed an atraumatic CSDH. The patient developed severe headaches in the hospital while being treated for febrile neutropenia. He remained neurologically intact. MMA embolization was undertaken due to the morbidity and mortality risks associated with surgery and the high risk of recurrence due to severe thrombocytopenia. At 2 months post-procedure the patient was asymptomatic and there was almost complete resolution of the hematoma. LESSONS: Thrombocytopenia in the presence of a CSDH is becoming increasingly common. This case highlights the particular role of MMA embolization in patients with severe thrombocytopenia where surgery carries high morbidity and increased mortality.

Deep Brain Stimulation and Swimming Performance: A Randomized Within-Person Crossover Study.

OBJECTIVE: To determine whether deep brain stimulation (DBS) causes swimming impairment, we systematically compared swimming ability between DBS on vs off in 18 patients. METHODS: We conducted a randomized blinded crossover study, comparing swimming ability between DBS on vs off, within participants. Participants swam 3 laps of front crawl and 3 laps of breaststroke. Prespecifed primary outcomes were proportion of lap completed, lap time, and Aquatic Skills Proficiency Assessment (ASPA) score. Prespecified secondary outcomes were a qualitative description of marked changes observed. RESULTS: Eighteen participants with Parkinson disease (n = 13), essential tremor (n = 3), Tourette syndrome (n = 1), or posttraumatic brain injury proximal tremor (n = 1), treated with posterior subthalamic area (n = 15) or globus pallidus interna (n = 3) DBS were assessed. There was no significant effect of DBS on/off status on any outcome measure for front crawl or breaststroke. Three participants showed changes in both qualitative and quantitative assessments. Of these, 1 participant displayed reduction in swimming ability: impairment in all outcomes with DBS on, normalizing with DBS off (the same individual as previously reported). The participant displayed difficulty coordinating limb movement as well as postural control. Two participants showed improvements in lap time and ASPA scores with DBS on. CONCLUSION: Overall DBS did not impair swimming performance, although 1 patient demonstrated a stimulation-induced drowning hazard. There were no anatomic or clinical features unique to the individual with swimming impairment. Patients should be warned about the possibility of DBS-induced drowning hazard and should swim with capable supervision after DBS. CLASSIFICATION OF EVIDENCE: This study provides Class IV evidence that for patients with implanted DBS electrodes, the stimulation on condition, compared with stimulation off, did not significantly impair swimming performance. A formal assessment of unblinding would have been helpful.

The Magnetic Resonance Imaging (MRI)-Directed Implantable Guide Tube Technique: Accuracy and Applications in Deep Brain Stimulation.

OBJECTIVE: The magnetic resonance imaging (MRI)-directed implantable guide tube technique allows for direct targeting of deep brain structures without microelectrode recording or intraoperative clinical assessment. This study describes a 10-year institutional experience of this technique including nuances that enable performance of surgery using readily available equipment. METHODS: Eighty-seven patients underwent deep brain stimulation surgery using the guide tube technique for Parkinson disease (n = 59), essential tremor (n = 16), and dystonia (n = 12). Preoperative and intraoperative MRI was analyzed to measure lead accuracy, volume of pneumocephalus, and the ability to safely plan a trajectory for multiple electrode contacts. RESULTS: Mean target error was measured to be 0.7 mm (95% confidence interval [CI] 0.6-0.8 mm) in the anteroposterior plane, 0.6 mm (95% CI 0.5-0.7 mm) in the mediolateral plane, and 0.8 mm (95% CI 0.7-0.9 mm) in the superoinferior plane. Net deviation (Euclidean error) from the planned target was 1.3 mm (95% CI 1.2-1.4 mm). Mean intracranial air volume per lead was 0.2 mL (95% CI 0.1-0.4 mL). In total, 52 patients had no intracranial air on postoperative imaging. In all patients, a safe trajectory could be planned to target for multiple electrode contacts without violating critical neural structures, the lateral ventricle, sulci, or cerebral blood vessels. CONCLUSIONS: The MRI-directed implantable guide tube technique is a highly accurate, low-cost, reliable method for introducing deep brain electrodes. This technique reduces brain shift secondary to pneumocephalus and allows for whole trajectory planning of multiple electrode contacts.

Endonasal endoscopic resection of suprasellar craniopharyngioma: A retrospective single-center case series.

INTRODUCTION: Endoscopic endonasal approach (EEA) has recently been proposed as an option for resection of primary and recurrent suprasellar craniopharyngioma. However, surgical outcome has not yet been fully evaluated, especially in regards to recurrent cases. METHODS: We analysed our institution (Sir Charles Gairdner University Hospital, Perth, Australia) case-series retrospectively. There were 16 patients operated through an endonasal endoscopic approach from February 2014 to February 2019 for suprasellar craniopharyngiomas. There were 14 primary, and two recurrent lesions. Extent of resection, complications, visual and endocrinological outcomes are presented. RESULTS: Mean age of the patients was 42.9 ± 19.3 years old, with 56% female. The most common clinical symptoms were headaches (9 patients, 56%) and bi-temporal hemianopsia (9 patients, 56%), followed by unilateral optic neuropathy (5 cases, 31%), memory loss (1 case, 6%), hydrocephalus (1 case, 6%), delayed growth and puberty (1 case, 6%), and secondary amenorrhoea (1 case, 6%). Only two cases (12%) initially presented with normal visual function. Gross total resection (GTR) was achieved in 10/16 patients (62.5%), with subtotal resection (STR) in the remainder. Visual symptoms improved in 13/16 patients (81%) and remained unchanged in 3/16 patients (19%). Most common complications included new endocrinological deficit in nine patients (56%), mostly diabetes insipidus, and cerebrospinal fluid leak requiring a new intervention in three patients (19%). There was one mortality case (complicated meningitis, stroke and vasospasm). Mean follow-up time was 22.05 ± 14 months and three patients (19%) had a recurrence of the disease during this period and were referred for radiation therapy. CONCLUSION: Endonasal endoscopic approach is a safe and effective surgical option for both primary and recurrent suprasellar craniopharyngiomas.

Prophylactic levetiracetam-induced pancytopenia with traumatic extra-dural hematoma: Case report.

BACKGROUND: Pancytopenia has only rarely been reported with Levetiracetam use. It is a potentially life threatening adverse effect that requires cessation of therapy. CASE DESCRIPTION: We describe a case of an otherwise well thirty-two-year-old man who underwent an emergent craniotomy for evacuation of a traumatic extra-dural haematoma. Post-operatively, he developed pancytopenia which corrected with cessation of levetiracetam. CONCLUSION: This report aims to increase awareness of this rare side effect and reiterates the judicious use of prophylactic levetiracetam in brain trauma.

Endoscopic Fenestration of Cavum Septum Pellucidum Cysts to the Third Ventricle: Technical Note.

BACKGROUND AND IMPORTANCE: Cavum septum pellucidum (CSP) and cavum vergae (CV) cysts are common incidental findings on imaging studies. However, they may rarely present with symptoms related to the obstruction of the foramen of Monro by the cyst leaflets. There is no consensus regarding the management of symptomatic CSP and CV cysts. We present an original transcavum interforniceal endoscopic fenestration technique. The step-by-step surgical procedure and two illustrative cases are presented. CLINICAL PRESENTATION: A 31-yr-old male and a 24-yr-old woman presented with symptomatic CSP and CV cysts. For both patients, neuronavigation was used to plan the procedure. An endoscope was introduced into the cyst through a right frontal burr-hole. After an examination of the endoscopic anatomy, a communication between the cyst and the third ventricle was performed using an endoscopic forceps. In both cases, directly after the fenestration, cerebrospinal fluid (CSF) passed through the communication, and the collapse of the cyst was appreciated. Symptoms were relieved in both patients, and neuropsychological assessment improved. Postoperative imaging showed a reduction in the cyst bulge, and patent foramen of Monro. CONCLUSION: Endoscopic fenestration of CSP and CV cysts to the third ventricle through an interforniceal navigated approach is a feasible and efficient surgical procedure. Theoretical advantages include a single tract through noneloquent brain, a perpendicular trajectory to the membrane for fenestration, and a large CSF space beyond the fenestration point.

Modulation of saccades in humans by electrical stimulation of the posterior subthalamic area.

OBJECTIVE: Posterior subthalamic area (PSA) deep brain stimulation (DBS) targeting the zona incerta (ZI) is an emerging treatment for tremor syndromes, including Parkinson's disease (PD) and essential tremor (ET). Evidence from animal studies has indicated that the ZI may play a role in saccadic eye movements via pathways between the ZI and superior colliculus (incerto-collicular pathways). PSA DBS permitted testing this hypothesis in humans. METHODS: Sixteen patients (12 with PD and 4 with ET) underwent DBS using the MRI-directed implantable guide tube technique. Active electrode positions were confirmed at the caudal ZI. Eye movements were tested using direct current electrooculography (EOG) in the medicated state pre- and postoperatively on a horizontal predictive task subtending 30°. Postoperative assessments consisted of stimulation-off, constituting a microlesion (ML) condition, and high-frequency stimulation (HFS; frequency = 130 Hz) up to 3 V. RESULTS: With PSA HFS, the first saccade amplitude was significantly reduced by 10.4% (95% CI 8.68%-12.2%) and 12.6% (95% CI 10.0%-15.9%) in the PD and ET groups, respectively. With HFS, peak velocity was reduced by 14.7% (95% CI 11.7%-17.6%) in the PD group and 27.7% (95% CI 23.7%-31.7%) in the ET group. HFS led to PD patients performing 21% (95% CI 16%-26%) and ET patients 31% (95% CI 19%-38%) more saccadic steps to reach the target. CONCLUSIONS: PSA DBS in patients with PD and ET leads to hypometric, slowed saccades with an increase in the number of steps taken to reach the target. These effects contrast with the saccadometric findings observed with subthalamic nucleus DBS. Given the location of the active contacts, incerto-collicular pathways are likely responsible. Whether the acute finding of saccadic impairment persists with chronic PSA stimulation is unknown.

Neobladder Obstruction: A Non-ischemic Cause for Hepatic Portal Venous Gas: Case Report.

Hepatic portal venous gas (HPVG) is a rare ominous radiological sign usually indicative of mesenteric ischemia. Increased detection of HPVG has been associated with a growing number of non-ischemic causes. A 64-year-old gentleman following radical cystectomy and neobladder formation developed clinical signs suggestive of bowel obstruction. HPVG was demonstrated on abdominal imaging. Urgent laparotomy revealed no evidence of ischemia. We hypothesize an obstructed neobladder permitted gas to enter the mesenteric circulation. The patient made a complete recovery with supportive management.

Drowning hazard with deep brain stimulation: case report.

The caudal zona incerta target within the posterior subthalamic area is an investigational site for deep brain stimulation (DBS) in Parkinson disease (PD) and tremor. The authors report on a patient with tremor-predominant PD who, despite excellent tremor control and an otherwise normal neurological examination, exhibited profound difficulty swimming during stimulation. Over the last 20 years, anecdotal reports have been received of 3 other patients with PD who underwent thalamic or pallidal lesioning or DBS surgery performed at the authors' center and subsequently drowned. It may be that DBS puts patients at risk for drowning by specifically impairing their ability to swim. Until this finding can be further examined in larger cohorts, patients should be warned to swim under close supervision soon after DBS surgery.