Hiccups Attributable to Syringobulbia and/or Syringomyelia Associated with a Chiari I Malformation: Case Report

Neurosurgery ◽  
2004 ◽  
Vol 54 (1) ◽  
pp. 224-227 ◽  
Author(s):  
Toshitaka Seki ◽  
Kazutoshi Hida ◽  
JangBo Lee ◽  
Yoshinobu Iwasaki
Keyword(s):  
Magnetic Resonance Imaging ◽  
Magnetic Resonance ◽  
Foramen Magnum ◽  
Chiari I Malformation ◽  
Neurological Deficits ◽  
Resonance Imaging ◽  
Foramen Magnum Decompression ◽  
Chiari I ◽  
Cerebellar Symptoms ◽  
Intractable Hiccups

Abstract OBJECTIVE AND IMPORTANCE Approximately 20 to 50% of patients with syringomyelia associated with Chiari malformations exhibit cranial nerve or cerebellar symptoms. However, hiccups represent a rare clinical manifestation of this disorder. We report a case of intractable hiccups resulting from syringobulbia associated with a Chiari I malformation, which was successfully treated with foramen magnum decompression. CLINICAL PRESENTATION We report the case of a patient who presented with syringomyelia and syringobulbia associated with a Chiari I malformation, manifested as intractable hiccups and neurological deficits. Magnetic resonance imaging scans demonstrated syringobulbia in the dorsal medullary region and a large cervical syrinx from C2 to C6–C7, associated with a Chiari I malformation. INTERVENTION Foramen magnum decompression and a C1 laminectomy were performed. One month later, the intractable hiccups disappeared and the neurological symptoms demonstrated improvement. CONCLUSION Postoperative magnetic resonance imaging scans demonstrated enlargement of the subarachnoid space in the posterior fossa and disappearance of the syringobulbia. There has been no recurrence of intractable hiccups and syringobulbia in 6 months after surgery. Magnetic resonance imaging of the brainstem is an important diagnostic procedure for intractable hiccups, because syringobulbia associated with a Chiari malformation represents a surgically treatable disorder, although the incidence is low.

Association of Chiari I Malformation, Mental Retardation, Speech Delay, and Epilepsy: A Specific Disorder?

Neurosurgery ◽  
2001 ◽  
Vol 49 (5) ◽  
pp. 1099-1104 ◽  
Author(s):  
Salvatore Grosso ◽  
Roberto Scattolini ◽  
Galluzzi Paolo ◽  
Rosanna Maria Di Bartolo ◽  
Guido Morgese ◽  
...  
Keyword(s):  
Magnetic Resonance Imaging ◽  
Mental Retardation ◽  
Magnetic Resonance ◽  
Incidental Finding ◽  
Foramen Magnum ◽  
Chiari I Malformation ◽  
Speech Delay ◽  
Resonance Imaging ◽  
Cavum Septum Pellucidum ◽  
Chiari I

ABSTRACT OBJECTIVE The Chiari I malformation is defined as tonsillar herniation of at least 3 to 5 mm below the foramen magnum. Although Chiari I malformation is considered to derive from a mesodermal disorder resulting in underdevelopment of the posterior fossa relative to its content, evidence for a possible heterogeneous etiology also has been reported. The aim of the present study is to elucidate the relationship between Chiari I malformation and mental retardation, speech delay, and epilepsy to consider a possible specific pathogenetic background. METHODS Thirty-five patients with Chiari I malformations were identified by use of magnetic resonance imaging during a period between 1993 and 1999. The study consisted of nine patients (four boys and five girls) who were affected by mental retardation, speech delay, and epilepsy. All patients underwent electroencephalography and brain and cervical spine magnetic resonance imaging. RESULTS All patients were mentally retarded with a mean intelligence quotient of 50. Seven patients had a positive history for speech delay, and five were epileptic. Electroencephalograms demonstrated abnormalities in seven patients. The mean tonsillar displacement was 10.1 mm. A thin corpus callosum and a wide cavum septum pellucidum were present in three patients. Neither hydromyelia nor scoliosis was observed. No correlation between the degree of the ectopia and clinical manifestation was noted. CONCLUSION The association of Chiari I malformation with epilepsy, speech delay, and mental retardation may not be a mere incidental finding but may be a marker for a different pathogenetic background.

SYMPTOMATIC CHIARI TYPE I MALFORMATION AFTER RADIATION THERAPY IN AN INFANT

Neurosurgery ◽  
2007 ◽  
Vol 60 (4) ◽  
pp. E782-E782 ◽  
Author(s):  
Caitlin E. Hoffman ◽  
Eric Lis ◽  
Suzanne L. Wolden ◽  
Mark M. Souweidane
Keyword(s):  
Magnetic Resonance Imaging ◽  
Radiation Therapy ◽  
Magnetic Resonance ◽  
Foramen Magnum ◽  
Chiari I Malformation ◽  
Type I ◽  
Malignant Rhabdoid Tumor ◽  
Resonance Imaging ◽  
Anterior Cranial Base ◽  
Chiari I

Abstract OBJECTIVE One previous case report has described the development of a Chiari I malformation in a child after fractionated radiation therapy to the anterior cranial base. The authors present an additional case of a 17-month-old girl treated for an isolated, malignant, rhabdoid tumor of the right neck with neoadjuvant chemotherapy, surgical exploration, and radiation therapy. CLINICAL PRESENTATION The patient developed a Chiari I malformation and cervical syringomyelia 1.5 years after the treatment. The patient experienced two episodes of unresponsiveness before diagnosis, and the malformation was then identified on magnetic resonance imaging scans. INTERVENTION Suboccipital decompressive craniectomy, and C1 laminectomy with autologous duraplasty were performed. RESULTS Postoperative magnetic resonance imaging scans revealed reconstitution of cerebrospinal fluid at the foramen magnum and ascent of the cerebellar tonsils. The patient remains asymptomatic 5.5 years after treatment. CONCLUSION The importance of this association is discussed in the context of the increasing use of radiation therapy in young children and infants.

scholarly journals Chiari I Malformation Associated with Turner Syndrome

2017 ◽  
Vol 08 (02) ◽  
pp. 277-280 ◽  
Author(s):  
Kamble Jayaprakash Harsha ◽  
Jeevan S. Nair
Keyword(s):  
Magnetic Resonance Imaging ◽  
Magnetic Resonance ◽  
Turner Syndrome ◽  
Genetic Disease ◽  
Clinical Presentation ◽  
Chiari I Malformation ◽  
Resonance Imaging ◽  
Cough Headache ◽  
First Case ◽  
Chiari I

ABSTRACTTurner syndrome (TS) is a rare genetic disease due to the absence of one X chromosome. Patients with TS have more subtle neurological/neuropsychiatric problems, while headache is an uncommon clinical presentation which needs attention. We report a 12-year-old child presenting with typical cough headache. Her magnetic resonance imaging revealed Chiari I malformation associated with TS. To the best of our knowledge, Chiari I malformation associated with TS is not described in literature. We report the first case of TS associated with Chiari I malformation. Interestingly, Chiari I malformation is also associated with Noonan’s syndrome, which is a close morphological mimicker of TS, raising the possibility of sharing similar pathogenesis in both conditions.

scholarly journals Surgical Experience in Pediatric Patients with Chiari-I Malformations Aged ≤18 Years

2019 ◽  
Vol 10 (01) ◽  
pp. 85-88 ◽  
Author(s):  
Ghanshyam Das Singhal ◽  
Shakti Singhal ◽  
Gunjan Agrawal ◽  
Deepti Singhal ◽  
Vipin Arora
Keyword(s):  
Pediatric Patients ◽  
Foramen Magnum ◽  
Chiari I Malformation ◽  
Neurological Deficits ◽  
Resonance Imaging ◽  
Surgical Experience ◽  
Limb Weakness ◽  
Lower Limb Weakness ◽  
Chiari I

ABSTRACT 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. There were 21 female children (42%) and 29 male children (58%), with a female-to-male ratio of 1:1. At the last follow-up, oropharyngeal symptoms were improved in 33% (n = 3/9). Headache/neck/back pain improved in 69.56% of children (n = 16/23). Upper-extremity pain/weakness/numbness improved in 73.91% of children (n = 17/23). Ataxia improved in 66.66% of children (n = 4/6). Lower-limb weakness/hyperreflexia improved in 83.33% of children (n = 5/6). At follow-up, magnetic resonance imaging for patients with syrinx was available for 75% of patients (n = 30/50) and not available for 25% of patients (n = 10/40). Syrinx was diminished in size or resolved in 66.33% of patients (n = 19/30) and the remaining was same for 36.66% of patients (n = 11/30). Conclusions: The main goal of surgery is to arrest the progression of neurological deficits. Foramen magnum decompression with a lax duroplasty is the surgical procedure of choice.

Spontaneous Resolution of Chiari I Malformation and Syringomyelia: Case Report and Review of the Literature

Neurosurgery ◽  
2001 ◽  
Vol 48 (3) ◽  
pp. 664-667 ◽  
Author(s):  
Jörg Klekamp ◽  
Giorgio Iaconetta ◽  
Madjid Samii
Keyword(s):  
Magnetic Resonance Imaging ◽  
Cerebrospinal Fluid ◽  
Case Report ◽  
Foramen Magnum ◽  
Chiari I Malformation ◽  
Spontaneous Resolution ◽  
Magnetic Resonance Imaging Scan ◽  
Resonance Imaging ◽  
Chiari I ◽  
Resonance Imaging Scan

Abstract OBJECTIVE AND IMPORTANCE Indications for surgery and the surgical technique of foramen magnum decompression for patients with Chiari I malformation and syringomyelia are controversial issues. This case report supports the view that observation may be adequate for patients without progressive symptoms or with mild clinical symptoms. CLINICAL PRESENTATION A 37-year-old woman presented with a 3-month history of burning dysesthesias and hypesthesia in her right arm. A neurological examination revealed hypesthesia in the right trigeminal distribution. A magnetic resonance imaging scan revealed a Chiari I malformation with syringomyelia between C2 and T2. No hydrocephalus was observed. CLINICAL COURSE Because the patient's symptoms regressed spontaneously, surgery was not performed. Thirty-two months after her initial examination, the patient was asymptomatic. A second magnetic resonance imaging scan was obtained, which demonstrated complete spontaneous resolution of the Chiari I malformation and syringomyelia. CONCLUSION We attribute the regression of the patient's symptoms to spontaneous recanalization of cerebrospinal fluid pathways at the foramen magnum, which most likely was due to rupture of the arachnoid membranes that had obstructed cerebrospinal fluid flow.

scholarly journals Analysis of magnetic resonance imaging–based blood and cerebrospinal fluid flow measurements in patients with Chiari I malformation: a system approach

2001 ◽  
Vol 11 (1) ◽  
pp. 1-10 ◽  
Author(s):  
Noam Alperin ◽  
Kirti Kulkarni ◽  
Ben Roitberg ◽  
Francis Loth ◽  
Naren K. Pandian ◽  
...  
Keyword(s):  
Magnetic Resonance Imaging ◽  
Magnetic Resonance ◽  
Mr Imaging ◽  
Cardiac Cycle ◽  
Chiari I Malformation ◽  
Intracranial Volume ◽  
Resonance Imaging ◽  
Csf Flow ◽  
Chiari I ◽  
Systolic Phase

Object A pilot study was performed to assess noninvasively the change in intracranial compliance (ICC) and intracranial pressure (ICP) in patients with Chiari I malformation who undergo foramen magnum decompression. The working hypothesis was that the main effect of the decompressive surgery is a change in ICP. Noninvasive cine phase-contrast magnetic resonance (MR) imaging is a motion-sensitive dynamic MR imaging technique that allows for visualization and quantitation of tissue motion and flow. The authors' group has used dynamic phase-contrast MR imaging to visualize and quantify pulsatile blood and cerebrospinal fluid (CSF) flow in the craniospinal system. Methods A system approach has been used to characterize the hemodynamic–hydrodynamic coupling in the craniospinal system and to derive measures for ICC and ICP. Magnetic resonance imaging–based ICC and ICP values are derived from the ratio of the volume and pressure changes that occur naturally during each cardiac cycle. The authors conducted a prospective study of four patients, three of whom were studied before and after decompressive surgery; significant change in MR imaging–derived ICC and ICP values was documented in only one of the three surgically treated patients. A significant change in the dynamics of the intracranial volume change (ICVC) during the cardiac cycle, however, was observed in all three patients. In healthy individuals the ICVC waveform usually consists of the following sequence: monotonic increase in intracranial volume (ICV) during the systolic phase due to increased blood inflow, monotonic decrease in ICV caused by the onset of CSF outflow into the spinal canal, and increase in the venous outflow. A nonmonotonic decline in the ICVC waveform has been observed in all patients with headaches, and a relatively normal waveform was found in those without headaches or whose headaches were resolved or alleviated by the surgery. A “partial-valve” mechanism is proposed as an explanation for the abnormal ICVC dynamics. The monotonic decline in ICVC is interrupted by a “premature” reduction in the CSF outflow. This may be caused by a displacement of the hindbrain into the cervical spinal canal during the systolic phase. This obstructs the CSF flow at the later part of the systolic phase such that the ICV does not continue its gradual decline. Postsurgery, the ICVC waveforms presented a more normal-appearing ICVC dynamics profile. Conclusions Magnetic resonance imaging measurement of transcranial CSF and blood flow may lead to a better understanding of the pathophysiology of Chiari malformations and may prove to be an important diagnostic tool for guiding for the treatment of patients with Chiari I malformation.

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