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Clin Neurol Neurosurg 2019 Apr 1;181:58-63. Epub 2019 Apr 1.

Department of Neurosurgery, The First Affiliated Hospital of Zhengzhou University, Zhengzhou, Henan province, 450052, PR China; Key laboratory of Neurosurgical Diseases, The First Affiliated Hospital of Zhengzhou University, Jianshe East Road, NO 1, Zhengzhou, Henan Province, 450052, PR China. Electronic address:

Objective: Syringomyelia was predominantly caused by Chiari malformation or intramedullary ependymoma. The goal of this study was to identify factors related to clinical outcomes and spinal hemangioblastoma (SH)-induced syringomyelia formation in a single series of patients.

Patient And Methods: Thirty-eight patients with SH were treated with microsurgery from January 2013 to December 2018. Read More

J Surg Case Rep 2019 Apr 12;2019(4):rjz082. Epub 2019 Apr 12.

Neurosurgery, Royal Hallamshire Hospital, Glossop Road, Sheffield S10 2JF, UK.

We present an unusual presentation of holocord syrinx. A 4-year-old boy presenting a 2-week history of right sided foot drop. An MRI revealed a holocord secondary to a chiari one malformation. Read More

Neurosurgery 2019 May;84(5):E270

Introduction: Chiari malformation I (CM-1) is a posterior fossa structural abnormality, characterized by caudal displacement of cerebellar tonsils, through the foramen magnum.Posterior fossa decompression (PFD), involving a suboccipital craniectomy and C1 laminectomy, remains the definitive treatment. Both PFD alone (PFDO) and PFD with additional duraplasty (PFDD) are used in surgical practices. Read More

Clinics (Sao Paulo) 2019 8;74:e653. Epub 2019 Apr 8.

Hospital Sao Marcos, Teresina, PI, BR.

Basilar invagination (BI) and Chiari malformation type I (CM-I) are very important anomalies that introduce instability and compression in the occipitocervical transition region and have complex clinical characteristics. These anomalies vary according to the affected structures. The present study revises current knowledge regarding the anatomy, anatomo-physiology, clinical manifestations, and radiological findings of these entities and the associated surgical treatment approaches. Read More

Asian Spine J 2019 Apr 2. Epub 2019 Apr 2.

Department of Neurosurgery, Hokkaido University Graduate School of Medicine, Sapporo, Japan.

Study Design: Retrospective cohort study.

Purpose: To investigate the correlation between the syrinx morphology and neuropathic pain caused by syringomyelia associated with Chiari I malformation.

Overview Of Literature: Neuropathic pain caused by syringomyelia is refractory and markedly impairs the patient. Read More

BMJ 2019 Apr 8;365:l1159. Epub 2019 Apr 8.

Department of Paediatric Neurosurgery, John Radcliffe Hospital, Oxford University Hospitals NHS Foundation Trust, Oxford OX3 9DU, UK.

Acta Neurochir (Wien) 2019 Apr 6. Epub 2019 Apr 6.

Institute of Neuroscience and Physiology, The Sahlgrenska Academy, University of Gothenburg, Gothenburg, Sweden.

Objective: To assess the neuropsychological (NP) functioning and quality of life (QOL) before and 3 months after surgery on adults with Chiari I malformation (CMI).

Patients And Methods: All adult patients who had been diagnosed with CMI were invited to participate. Those who participated were assessed using a Hospital Anxiety and Depression scale (HAD) and NP examinations. Read More

J Clin Sleep Med 2019 Apr 2. Epub 2019 Apr 2.

Abstract: Arnold Chiari malformation (ACM) is the most common cause of central sleep apnea (CSA) in otherwise healthy children. Although there are several case reports and series reported on this topic, there are limited descriptions of the long-term course of these children after the surgical interventions. Posterior fossa decompression surgery to relieve pressure of the herniating cerebellum on the brainstem is generally thought to significantly improve CSA in most cases, however, there are very limited data on the natural course of CSA in children following decompression surgery. Read More

Neuroimaging Clin N Am 2019 May 26;29(2):xv. Epub 2019 Feb 26.

Department of Radiology, Michigan State University, Michigan State University Health Team, 846 Service Road, East Lansing, MI 48824, USA. Electronic address:

Neuroimaging Clin N Am 2019 May 20;29(2):243-253. Epub 2019 Feb 20.

Department of Radiology, Harvard Medical School, Beth Israel Deaconess Medical Center, 1 Deaconess Road, Boston, MA 02215, USA. Electronic address:

Headache is a common symptom in patients with Chiari I malformation (CMI), characterized by 5 mm or greater cerebellar tonsillar herniation below foramen magnum. Radiologists should be aware of the different types of headaches reported by CMI patients and which headache patterns are distinctive features of the diagnosis. A methodical imaging strategy is required to fully assess a CMI patient to exclude secondary causes of tonsillar herniation such as intracranial hypotension or associated conditions such as syrinx. Read More

J Neurosurg Pediatr 2019 Mar 29:1-4. Epub 2019 Mar 29.

1Geisel School of Medicine at Dartmouth, Hanover; and.

Suboccipital craniectomy with duraplasty is a commonly performed procedure for children with symptomatic Chiari malformation type I (CM-I). Several dural substitutes are used for duraplasty, ranging from pericranium to synthetic materials. When available, autologous pericranium is often preferred due to its low cost, performance in obtaining a watertight closure, ease of suturing, and absence of immune reaction. Read More

Childs Nerv Syst 2019 Mar 27. Epub 2019 Mar 27.

Department of Pediatric Neurosurgery, Hôpital Femme Mère Enfant, 32 Avenue du Doyen Jean Lépine, 69677, Lyon Cedex, France.

Background: Postnatal closure of a myelomeningocele remains the standard of care in many countries. The prenatal closure has given hope for decreasing the damage to the neural placode and has challenged classic management. However, this technique presents potential sources of complications. Read More

Vet J 2019 Apr 1;246:92-97. Epub 2019 Feb 1.

Department of Veterinary Clinical Sciences, Faculty of Health and Medical Sciences, University of Copenhagen, Dyrlaegevej 16, 1870 Frederiksberg C, Denmark.

It is assumed that Cavalier King Charles spaniels with Chiari-like malformation and syringomyelia experience central neuropathic pain. An association between spinal cord parenchymal lesions and specific clinical signs (e.g. Read More

Cephalalgia 2019 Mar 20:333102419839793. Epub 2019 Mar 20.

1 Headache Unit, University Hospital of Valladolid, Valladolid, Spain.

Background: Epicrania fugax is included in the appendix of the International Classification of Headache Disorders and is characterized as recurrent brief attacks of linear or zigzag pain moving across the cranial surface, commencing and terminating in the distribution of different nerves. We present a new case of epicrania fugax in which the headache was the presenting symptom of a cerebellar abscess.

Case Report: We present a 58-year-old woman with prior history of Chiari I malformation who underwent suboccipital craniectomy. Read More

Vet Radiol Ultrasound 2019 Mar 19. Epub 2019 Mar 19.

Department of Clinical Sciences, Cornell University, College of Veterinary Medicine, Ithaca, New York.

Syringobulbia is a pathologic condition characterized by one or more fluid-filled cavities within the brainstem. This retrospective case series describes observations in eight dogs with syringobulbia diagnosed during MRI. All dogs were adult, small-breed dogs with concurrent syringomyelia and neurologic deficits localized to sites rostral to the spinal cord, which cannot be explained by syringomyelia (eg, six dogs had vestibular signs). Read More

Anesth Prog 2019 ;66(1):37-41

Department of Dental Anesthesiology, Nippon Dental University School of Life Dentistry, Tokyo, Japan.

Type II Arnold-Chiari malformation (ACM) is an abnormality in which the cerebellum, pons, and medulla oblongata are displaced downward into the spinal cord. Type II ACM is often complicated by respiratory depression, sleep-disordered breathing, and deglutition disorder as a result of medullary dysfunction and impairment of the lower cranial nerves. Bending and stretching of the neck is restricted, and anesthetic management is problematic in patients with the disorder. Read More

Plast Reconstr Surg Glob Open 2019 Feb 12;7(2):e2090. Epub 2019 Feb 12.

Department of Ophthalmology, Children's Hospital of Pittsburgh of UPMC, Pittsburgh, Pennsylvania.

Background: We report the incidence of Chiari malformation I (CMI) in a cohort of 377 patients with isolated sagittal synostosis (ISS), which is to the best of our knowledge the largest such series reported to date.

Methods: A retrospective review of patients seen at a single institution from 2007 to 2017 was completed. ISS, Chiari malformations (CMI and CMII) and hydrocephalus were diagnosed by a senior neuroradiologist (G. Read More

Pediatr Emerg Care 2019 Apr;35(4):e72-e75

Department of Neurosurgery, Rutgers Robert Wood Johnson Medical School, New Brunswick, NJ.

In this report, we describe a 13-year-old with opisthotonos as the presenting symptom of Chiari I malformation. This presentation is rare and has previously been reported only in infants. We describe the physical and radiologic findings, literature regarding Chiari malformation, and differential diagnosis of opisthotonos in this patient. Read More

J Small Anim Pract 2019 Mar 13. Epub 2019 Mar 13.

Dick White Referrals, Six Mile Bottom, Cambridgeshire CB8 0UH, UK.

Objectives: To describe the prevalence, clinical findings and predictors of disease in dogs with cervical hyperaesthesia.

Materials And Methods: Medical records of dogs referred for neurological investigation of cervical hyperaesthesia between 2009 and 2013 were retrospectively reviewed. Dogs were assigned to one of the following groups according to the final diagnosis: Non-Neurologic, Brain, Cervical Spine, Multifocal, and Chiari-like Malformation/Syringomyelia. Read More

J Craniofac Surg 2019 Mar 6. Epub 2019 Mar 6.

Department of Neurosurgery.

The fourth ventricle outlet obstruction (FVOO) is a rare but well-established cause of obstructive tetra-ventricular hydrocephalus, characterizing with dilatation or large cerebrospinal fluid collection of the foramina of Magendie and Luschka. In children, it is usually the consequence of posterior cerebral fossa malformations; while in adult, the occlusion is rather acquired than congenital, mostly linked to an inflammatory process, intraventricular hemorrhage, head trauma, brain tumors or Arnold-Chiari malformation. However, idiopathic FVOO is extremely rare, and only 6 such cases have been reported in the English literature. Read More

World Neurosurg 2019 Mar 1. Epub 2019 Mar 1.

Section of Neurosurgery, The University of Chicago Medicine, Chicago, Illinois, USA.

Objective: Adults with Chiari malformation type 1 (CM1) often report cognitive impairment. This cross-sectional study investigates the cognitive and emotional functioning of a sample of adults with CM1 who presented for neurosurgical evaluation prior to intervention.

Methods: A total of 36 participants (18 patients with CM1 and 18 healthy control subjects) completed a comprehensive neurocognitive battery of tests. Read More

Clin Radiol 2019 May 26;74(5):408.e19-408.e25. Epub 2019 Feb 26.

Academic Unit of Radiology, University of Sheffield, Sheffield, UK. Electronic address:

Aim: To describe a new finding in fetuses with Chiari 2 malformations recognised at in utero (iu) magnetic resonance imaging (MRI), specifically T2 prolongation (high signal) in the cerebellar vermis.

Materials And Methods: This was a prospective observational study of iuMRI studies performed at two time points on 20 fetuses with Chiari 2 malformations and 10 control fetuses at the same time points. High T2 signal in the cerebellar vermis was noted and correlated with posterior fossa dimensions was assessed. Read More

Am J Med Genet A 2019 May 17;179(5):792-796. Epub 2019 Feb 17.

Division of Medical Genetics, University of Utah, Salt Lake City, Utah.

The NONO gene encodes a nuclear protein involved in RNA metabolism. Hemizygous loss-of-function NONO variants have been associated with syndromic intellectual disability and with left ventricular noncompaction (LVNC). A two-year-old boy presented to the University of Utah's Penelope Undiagnosed Disease Program with developmental delay, nonfamilial features, relative macrocephaly, and dilated cardiomyopathy with LVNC and Ebstein anomaly. Read More

J Neurosci Rural Pract 2019 Jan-Mar;10(1):85-88

Department of Neurosurgery, Maharaja Agrasen Hospital, New Delhi, India.

Objective: The objective of this study was to retrospectively study Chiari I malformation patients (<18 years) treated surgically.

Materials And Methods: Chiari I malformation patients (<18 years) treated surgically at our institute were retrospectively studied.

Results: During the study period between January 1999 and June 2011, fifty patients, aged ≤18 years with Chiari malformation, were treated surgically and formed the basis for this series. Read More

Am J Med Genet A 2019 Apr 13;179(4):615-627. Epub 2019 Feb 13.

Clinical Genetics Service, Great Ormond Street Hospital, London, United Kingdom.

Mutations in the ERF gene, coding for ETS2 repressor factor, a member of the ETS family of transcription factors cause a recently recognized syndromic form of craniosynostosis (CRS4) with facial dysmorphism, Chiari-1 malformation, speech and language delay, and learning difficulties and/or behavioral problems. The overall prevalence of ERF mutations in patients with syndromic craniosynostosis is around 2%, and 0.7% in clinically nonsyndromic craniosynostosis. Read More

Neurochirurgie 2019 Feb 8. Epub 2019 Feb 8.

Service de neurochirurgie, hôpital Nord, 13015 Marseille, France.

Background: The Chiari malformation type I (CM-I) is the most commonly found type in adults. The efforts to further improve the treatment offered for these malformations are hampered by the existence of controversial methods and the absence of a uniform scoring system to evaluate clinical outcomes.

Objectives: The goal of our study is to analyze the clinical and radiological data concerning patients operated for CM and to expose surgical techniques. Read More

Acta Neurol Belg 2019 Feb 8. Epub 2019 Feb 8.

Department of Neurosurgery, University Hospitals, Leuven, Belgium.

Objective: Determining clinical and radiological characteristics, complication rates and outcome for patients undergoing posterior fossa decompression (PFD) and duraplasty for Chiari malformation type I (CM-I).

Methods And Materials: Retrospective, single-university hospital study of all PFDs for CM-I between January 1995 and December 2016.

Results: PFD was performed in 105 patients with CM-I (n = 105), of whom 62 suffered from associated syringomyelia and 37 were pediatric cases. Read More

Childs Nerv Syst 2019 May 6;35(5):879-882. Epub 2019 Feb 6.

Department of Surgery, Division of Neurosurgery, College of Health Sciences, University of Zimbabwe, P.O Box A178, Avondale, Harare, Zimbabwe.

Bobble-head doll syndrome is a rare movement disorder that is usually associated with lesions involving the third ventricle. It is characterised by stereotypical rhythmic up-and-down or side-to-side head movements. The pathophysiology and anatomical basis for this unusual manifestation is still a subject of intense scrutiny. Read More

Neurosurgery 2019 Jan 23. Epub 2019 Jan 23.

Division of Neuroscience, National Institutes of Health/National Institute of Neurological Disorders and Stroke, Bethesda, Maryland.

The management of Chiari I malformation (CMI) is controversial because treatment methods vary and treatment decisions rest on incomplete understanding of its complex symptom patterns, etiologies, and natural history. Validity of studies that attempt to compare treatment of CMI has been limited because of variable terminology and methods used to describe study subjects. The goal of this project was to standardize terminology and methods by developing a comprehensive set of Common Data Elements (CDEs), data definitions, case report forms (CRFs), and outcome measure recommendations for use in CMI clinical research, as part of the CDE project at the National Institute of Neurological Disorders and Stroke (NINDS) of the US National Institutes of Health. Read More

Insights Imaging 2019 Jan 28;10(1). Epub 2019 Jan 28.

Department of Radiodiagnosis, Pediatric Radiology section, Faculty of Medicine, Ain-Shams University, Abbasia, Cairo, 11657, Egypt.

CSF flow disorders are frequently encountered in children. The advent of MR technology with the emergence of new pulse sequences allowed better understanding of CSF flow dynamics. In this pictorial review, we aim to conduct a comprehensive review of the MR protocol used to study CSF flow disorders and to discuss the utility of each pulse sequence in the adopted protocol. Read More

World Neurosurg 2019 Jan 25. Epub 2019 Jan 25.

Spine Surgery, Drum Tower Hospital of Nanjing University Medical School, Nanjing, China. Electronic address:

Objective: To compare vertebral morphology among patients with adolescent idiopathic scoliosis (AIS), patients with Chiari I malformation (CMS)-associated scoliosis, and normal control subjects, with the goal of determining the role of anterior column overgrowth in the development of AIS.

Methods: One hundred and forty adolescent girls were enrolled (50 with AIS, 40 with CMS, and 50 control subjects). Thoracic computed tomography images were obtained for all subjects. Read More

Neurosurg Rev 2019 Jan 25. Epub 2019 Jan 25.

Department of Neurosurgery, Shinshu University School of Medicine, 3-1-1 Asahi, Matsumoto, 390-8621, Japan.

Several surgical procedures can be applied for syrinx associated with Chiari type 1 malformation; however, it remains controversial as to which approach is the most effective. Here, we evaluated the indications and limitations of foramen magnum decompression (FMD) with or without dural plasty. Forty patients with Chiari type 1 malformation were surgically treated and followed up for > 12 months. Read More

Eur J Paediatr Neurol 2019 Mar 14;23(2):333-337. Epub 2019 Jan 14.

Department of Paediatric Neurosciences, Royal Hospital for Sick Children, Edinburgh, UK.

Introduction: Neurosurgical treatment is recommended for symptomatic syringomyelia and the post-operative radiological resolution of the syringomyelia is associated with an improvement or at least stability of the patient's pre-operative symptoms.

Methods: We reviewed syringomyelia treatment in our centre over the last five years for clinical outcome, surgical complications, post operative MRI and long term symptom resolution.

Results: 50 cases of symptomatic syringomyelia underwent foramen magnum decompression and expansile watertight duroplasty. Read More

Medicine (Baltimore) 2019 Jan;98(4):e14232

College of Medicine, Qatar University, Doha, Qatar.

Rationale: Hydrocele of the canal of Nuck is a rare developmental disorder and represents of a homolog of hydrocele of spermatic cord in males. Hydrocele of the canal of Nuck is a very rare cause of inguinal swelling in female infants and children. It results from the failure of obliteration of the distal portion of evaginated parietal peritoneum within the inguinal canal, which forms a sac containing fluid. Read More

Zhonghua Yi Xue Za Zhi 2019 Jan;99(3):183-187

Department of Spinal Surgery, Drum Tower Hospital Clinical College of Nanjing Medical University, Nanjing 210008, China.

To evaluate the long term change of the cervical sagittal profile in adolescent Chiari malformation type Ⅰ (CMI)/syringomyelia undergoing posterior fossa decompression (PFD) and to further evaluate the correlation between the syrinx resolution and cervical sagittal profile. A retrospective radiographic study was performed in 32 adolescents undergoing PFD for CMI/syringomyelia from October 2011 to August 2015 with a minimum 2-year follow-up. There were 23 males and 9 females, with a mean age of (13. Read More

Acta Neurochir (Wien) 2019 Feb 22;161(2):367-370. Epub 2019 Jan 22.

Departments of Neurosurgery and Pediatric Neurosurgery, Tel Aviv Medical Center and Dana Children's Hospital Tel Aviv, Tel Aviv University, Tel Aviv, Israel.

Background: Syringo-subarachnoid shunt (SSS) is a valid method for the treatment of syringomyelia persisting after foramen magnum decompression (FMD) for Chiari I malformation.

Method: We give a brief overview on indication and outcome of SSS, followed by a detailed description of the surgical anatomy, and of the microsurgical technique. In particular, we highlight some key points for complication avoidance. Read More

J Neurosurg Pediatr 2019 Jan 18:1-2. Epub 2019 Jan 18.

Neurospine 2018 Oct 15. Epub 2018 Oct 15.

Department of Orthopedic Surgery, Spine Unit, Rigshospitalet, University of Copenhagen, Copenhagen, Denmark.

Objective: MRI-verified neural axis abnormality (NAA) has been described in adolescent idiopathic scoliosis (AIS) and several risk factors have been associated with the presence of NAA in AIS-patients. However, the clinical significance of these findings is not clear. The purpose of the present study was to determine the prevalence of NAAs in a large consecutive cohort of AIS patients and evaluate the clinical significance of previously proposed risk factors. Read More

Turk Neurosurg 2019 ;29(2):229-236

Mustafa Kemal University, School of Medicine, Department of Neurosurgery, Hatay, Turkey.

Aim: To compare the clinical and radiological results of dural splitting and duraplasty in patients with Chiari Type I Malformation.

Material And Methods: This study includes 113 adult patients with Chiari Type I malformation treated between 2009 and 2013. The patients were divided into two groups according to the surgical method (Group 1: dural splitting, Group 2: duraplasty). Read More

Vet Rec 2019 Jan 11;184(4):122. Epub 2019 Jan 11.

Faculty of Health and Medical Sciences, School of Veterinary Medicine, University of Surrey, Guildford, UK.

Modern interpretation of head conformation in the Cavalier King Charles spaniel (CKCS) has favoured a smaller, more exaggerated, brachycephalic type than originally described in the 1929 breed standard. Recent research studies identified brachycephaly and reduced hind cranium as two conformational (dysmorphic) features that increase risk for symptomatic Chiari-like malformation and secondary syringomyelia (SM). A prospective pilot study investigated the hypothesis that dysmorphic head features could be assessed visually and correlated with risk of SM. Read More

Neurosurg Rev 2019 Jan 9. Epub 2019 Jan 9.

Medical University of South Carolina, Charleston, SC, USA.

A great deal of literature has drawn attention to the "complex Chiari," wherein the presence of instability or ventral brainstem compression prompts consideration for addressing both concerns at the time of surgery. This report addresses the clinical and radiological features and surgical outcomes in a consecutive series of subjects with hereditary connective tissue disorders (HCTD) and Chiari malformation. In 2011 and 2012, 22 consecutive patients with cervical medullary syndrome and geneticist-confirmed hereditary connective tissue disorder (HCTD), with Chiari malformation (type 1 or 0) and kyphotic clivo-axial angle (CXA) enrolled in the IRB-approved study (IRB# 10-036-06: GBMC). Read More

J Clin Sleep Med 2019 Jan 15;15(1):89-99. Epub 2019 Jan 15.

Neurotraumatology and Neurosurgery Research Unit (UNINN), Vall d'Hebron University Hospital, Barcelona, Spain.

Study Objectives: The aim of this study is to generate and validate supervised machine learning algorithms to detect patients with Chiari malformation (CM) 1 or 1.5 at high risk of the development of sleep-related breathing disorders (SRBD) using clinical and neuroradiological parameters.

Methods: We prospectively included two independent datasets. Read More

Head Neck 2019 May 8;41(5):E71-E78. Epub 2019 Jan 8.

Department of Neurology, University of Massachusetts Medical School, Worcester, Massachusetts.

Background: Although sensory feedback is a vital regulator of deglutition, it is not comprehensively considered in the standard dysphagia evaluation. Difficulty swallowing secondary to sensory loss may be termed "sensory dysphagia" and may account for cases receiving diagnoses of exclusion, like functional or idiopathic dysphagia.

Methods And Results: Three cases of idiopathic dysphagia were suspected to have sensory dysphagia. Read More

J Craniofac Surg 2018 Dec 29. Epub 2018 Dec 29.

Department of Plastic, Reconstructive and Maxillo-Facial Surgery, and Burn Unit, Centro Hospitalar de São João, Porto Medical School, Alameda Professor Hernâni Monteiro, Porto, Portugal.

A 68-year-old woman, presented with a squamous cell carcinoma of the malar region, and underwent wide local excision. During her clinical examination, repetitive protrusion and intrusion of the tongue as well as stereotypic, abnormal movements of the mouth and lips were observed, in a pattern that resembled chewing, sucking or lip pursing; dyskinesias ceased when she was speaking or bringing food to the mouth. She was unaware of the movements and the tongue was observed to move similar to choreiform movements, while revealing a giant "snake-like" macroglossia. Read More

BMC Musculoskelet Disord 2019 Jan 5;20(1):10. Epub 2019 Jan 5.

Department of Orthopaedic Surgery, Peking Union Medical College Hospital, Chinese Academy of Medical Science and Peking Union Medical College, Beijing, 100730, China.

Background: Klippel-Trenaunay syndrome (KTS) is a rare congenital syndrome characterized by the triad of venous varicosities, capillary malformations and limb hypertrophy. However, KTS may rarely occur in combination with kyphoscoliosis.

Case Presentation: We presented an 18-year-old female with KTS and kyphoscoliosis. Read More

J Neurosurg Pediatr 2019 01 4:1-9. Epub 2019 Jan 4.

1Department of Neurosurgery and Division of Pediatric Neurosurgery, University of Alabama at Birmingham, Alabama; and.

OBJECTIVE There are sparse published data on the natural history of “benign” Chiari I malformation (CM-I)—i.e., Chiari with minimal or no symptoms at presentation and no imaging evidence of syrinx, hydrocephalus, or spinal cord signal abnormality. Read More

Cerebellum 2019 Jan 4. Epub 2019 Jan 4.

Department of Neurology, Seoul National University College of Medicine, Seoul, South Korea.

The mechanism of perverted vertical responses during horizontal head impulse tests (HITs) requires further elucidation. A 47-year-old woman with a Chiari malformation showed alternating skew deviation, downbeat nystagmus with an increasing slow phase velocity, impaired smooth pursuit, and upward ocular deviation during horizontal HITs and corrective downward saccades in the presence of normal bithermal caloric tests and intact tilt suppressions of the post-rotatory nystagmus. These findings suggest dysfunction of the inferior cerebellum including the tonsil, nodulus, and uvula. Read More

Childs Nerv Syst 2019 Apr 4;35(4):707-711. Epub 2019 Jan 4.

Department of Neurosurgery, China National Clinical Research Center for Neurological Diseases, Beijing Tiantan Hospital, Capital Medical University, Beijing, China.

Background: Shunt dependency syndrome is a rare long-term complication of cystoperitoneal (CP) shunting for intracranial arachnoid cysts, which is characterized by acute intracranial hypertension with normal-sized or small ventricles. Additionally, acquired Chiari type I malformations (ACIM) could be infrequently secondary to extrathecal shunt drainage of cerebrospinal fluid.

Case Report: We described a 12-year-old boy who developed shunt dependency syndrome following a CP shunting for treating a temporal arachnoid cyst. Read More

Acta Neurochir Suppl 2019;125:229-233

Neurosurgical Unit, Surgical Department, Azienda Ospedaliera SS Antonio e Biagio e Cesare Arrigo, Alessandria, Italy.

Background: The craniovertebral junction (CVJ) is often involved in a wide range of congenital, developmental and acquired pathologies that can create bony and ligamentous instability or cause direct compression on the medulla and cervical spine cord, resulting in significant impairment. Atlas assimilation is the most common malformation in the CVJ and can be frequently associated with basilar invagination (BI) and Chiari malformation (CM) type I. Posterior atlas assimilation more frequently leads to BI type II with a mass effect on neural structures but usually no signs of biomechanical instability. Read More

Acta Neurochir Suppl 2019;125:139-143

Department of Neurosurgery, Policlinico "A. Gemelli", University "Cattolica", Rome, Italy.

This paper has been edited for clarity, correctness and consistency with our house style. Please check it carefully to make sure the intended meaning has been preserved. If the intended meaning has been inadvertently altered by the editing changes, please make any corrections needed. Read More