Chiari I Malformation Redefined: Clinical and Radiographic Findings for 364 Symptomatic Patients

Milhorat, Thomas H.; Chou, Mike W.; Trinidad, Elizabeth M.; Kula, Roger W.; Mandell, Menachem; Wolpert, Chantelle M.; Speer, Marcy C.

doi:10.1097/00006123-199905000-00042

Cited by 1,149 publications

(1,085 citation statements)

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“…In our view, these two conditions – although each with its typical clinical and radiological characteristics (Friedman et al., 2013; Milhorat et al., 1999) – are both often characterized by some degree of reduced ICC as recently addressed (Frič & Eide, 2016). A particular aspect of these two conditions is that they are often present during early adulthood, that is, in much younger age than, for example, a typical case of iNPH.…”

Section: Discussionmentioning

confidence: 80%

“…Chiari malformation type 1 (CMI), characterized by idiopathic caudal ectopy of cerebellar tonsils into foramen magnum (Milhorat et al., 1999), and idiopathic intracranial hypertension (IIH) (Friedman, Liu, & Digre, 2013) are traditionally considered to be two distinct clinical entities, although some common clinical, radiological, and pathophysiological features have been noticed (Bejjani, 2003; Frič & Eide, 2016). In particular, disturbed cerebrospinal fluid circulation (CSF) resulting in reduced intracranial compliance (ICC) has been demonstrated as a characteristic feature of both CMI and IIH (Eide & Kerty, 2011; Frič & Eide, 2015, 2016).…”

Section: Introductionmentioning

confidence: 99%

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Cardiovascular risk factors in Chiari malformation and idiopathic intracranial hypertension

2017

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ObjectivesBoth Chiari malformation type 1 (CMI, i.e., the idiopathic caudal ectopy of cerebellar tonsils into foramen magnum) and idiopathic intracranial hypertension (IIH) are characterized by reduced intracranial compliance (ICC) due to disturbed circulation of cerebrospinal fluid (CSF). An increasing body of evidence links cardiovascular disease to CSF circulation disturbances. The aim of this study was to explore whether the prevalence of cardiovascular risk factors in patients with CMI or IIH is higher than in the general population.Materials and MethodsAmong the patients with CMI or IIH treated at our department during the period 2003–2014, we identified those with history of arterial hypertension (AH), myocardial infarction (MI), angina pectoris (AP), or diabetes mellitus (DM). For comparison with a control population, we retrieved information about the prevalence of AH, MI, AP, and DM among participants of the North‐Trøndelag Health Study 3 (HUNT3).ResultsData from 48 CMI and 52 IIH cases were available. Compared to data from the 42,461 individuals participating in the HUNT3, we found increased prevalence of DM in male CMI as well as female IIH cases, and of AH in female IIH cases. Body mass index (BMI) was significantly increased in both female and male IIH cases. Prevalence of MI and AP in the CMI and IIH cohorts was extremely low and therefore not further studied.ConclusionsThis study provided evidence of an increased prevalence of DM in male CMI as well as female IIH cases and of AH in female IIH cases. Although requiring further exploration, these findings point to AH and DM as potential risk factors in the pathophysiology of CMI and IIH.

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Section: Discussionmentioning

confidence: 80%

Section: Introductionmentioning

confidence: 99%

Cardiovascular risk factors in Chiari malformation and idiopathic intracranial hypertension

2017

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“…Many of the symptoms reported by fibromyalgia patients are identical to those reported by patients diagnosed with two well defined neurological disorders: Chiari 1 malformation or cervical myelopathy due to spinal stenosis (spondylotic cervical myelopathy) [1,13,29]. We previously reported the radiological and neurological findings in non-randomly selected patients with fibromyalgia, documenting the presence of cervical myelopathy and cervical spinal cord compression in a cohort of 270 patients [17].…”

Section: Introductionmentioning

confidence: 87%

Treatment of cervical myelopathy in patients with the fibromyalgia syndrome: outcomes and implications

et al. 2007

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Some patients with fibromyalgia also exhibit the neurological signs of cervical myelopathy. We sought to determine if treatment of cervical myelopathy in patients with fibromyalgia improves the symptoms of fibromyalgia and the patients' quality of life. A non-randomized, prospective, case control study comparing the outcome of surgical (n = 40) versus non-surgical (n = 31) treatment of cervical myelopathy in patients with fibromyalgia was conducted. Outcomes were compared using SF-36, screening test for somatization, HADS, MMPI-2 scale 1 (Hypochondriasis), and self reported severity of symptoms 1 year after treatment. There was no significant difference in initial clinical presentation or demographic characteristics between the patients treated by surgical decompression and those treated by non-surgical means. There was a striking and statistically significant improvement in all symptoms attributed to the fibromyalgia syndrome in the surgical patients but not in the non-surgical patients at 1 year following the treatment of cervical myelopathy (P £ 0.018-0.001, Chi-square or Fisher's exact test). At the 1 year follow-up, there was a statistically significant improvement in both physical and mental quality of life as measured by the SF-36 score for the surgical group as compared to the non-surgical group (Repeated Measures ANOVA P < 0.01). There was a statistically significant improvement in the scores from Scale 1 of the MMPI-2 and the screening test for somatization disorder, and the anxiety and depression scores exclusively in the surgical patients (Wilcoxon signed rank, P < 0.001). The surgical treatment of cervical myelopathy due to spinal cord or caudal brainstem compression in patients carrying the diagnosis of fibromyalgia can result in a significant improvement in a wide array of symptoms usually attributed to fibromyalgia with attendant measurable improvements in the quality of life. We recommend detailed neurological and neuroradiological evaluation of patients with fibromyalgia in order to exclude compressive cervical myelopathy, a potentially treatable condition.

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“…In his another study, 46% (88/190) of basilar invagination (BI) cases had AAD. In a series of 364 patients with symptomatic CMI, Milhorat et al [7] reported (BI) in 12%, atlas assimilation in 5.2%, and the Klippel-Feil anomaly in 4.9%. Such disparity has probably several reasons, such as different including criteria, surgeon's clinical experience and the use of more accurate preoperative examinations (CT and MRI).…”

Section: History Diagnostic Differential Diagnosis Epidemiologymentioning

confidence: 95%

Syringomyelia with irreducible atlantoaxial dislocation, basilar invagination and Chiari I malformation

Wang

Yan

et al. 2009

Eur Spine J

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A 27-year-old woman presented with bilateral weakness of her all extremities for 5 years. She had a spastic gait and was unable to ambulate without assistance. Neurologic examination revealed increased deep tendon reflexes and positive pathologic reflexes. Radiographs showed occipitalization of the atlas, C2-C3 congenital fusion and fixed atlantoaxial dislocation with an atlanto-dental interval of 10 mm. MRI demonstrated cervicomedullary junction (CMJ) compression from the odontoid, a Chiari type I malformation, and syringomyelia extending from the foramen magnum to C5. The patient underwent transoral atlantoaxial release followed by posterior internal fixation from the occiput to the axis, which resulted in a significant improvement in motor function in all extremities. Postoperative images showed anatomical reduction of the atlantoaxial joint. However, an MRI performed 8 days following surgery showed a new retro-odontoid pannus had developed that was compressing the spinal cord at CMJ. A follow-up CT scan performed at 6 months post-operatively demonstrated a solid bony fusion between the occiput and C2, while an MRI at that time showed complete resolution of the retro-odontoid soft tissue mass with correction of the Chiari I malformation, and resolution of the syringomyelia. Final follow-up at 2-years revealed an excellent clinical outcome.Keywords Syringomyelia Á Irreducible atlantoaxial dislocation Á Basilar invagination Á Surgery Á Transoral release Case presentationA 27-year-old woman presented with a 5-year history of progressive weakness and numbness that began in her right extremities. Her symptoms progressed rapidly for the first year and eventually her entire right side became involved. When admitted, she complained of an unsteady gait requiring an assistive device to ambulate, and lacked coordination in her hands (impossible to button up her shirt). Neurologic examination of the lower extremities revealed normal sensation to light touch, pain and temperature, while sensory dysfunction of the upper limbs was present. Symmetrical hyperreflexia, as well as positive Hoffman's and Babinski signs were present bilaterally. Weakness was present on both sides, with the right side being more severe. Her gait was wide-based, spastic, and unsteady. She also had limited range of motion on cervical

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Chiari I Malformation Redefined: Clinical and Radiographic Findings for 364 Symptomatic Patients

Cited by 1,149 publications

References 88 publications

Cardiovascular risk factors in Chiari malformation and idiopathic intracranial hypertension

Cardiovascular risk factors in Chiari malformation and idiopathic intracranial hypertension

Treatment of cervical myelopathy in patients with the fibromyalgia syndrome: outcomes and implications

Syringomyelia with irreducible atlantoaxial dislocation, basilar invagination and Chiari I malformation

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