Tonsillar ectopia and headaches

doi:10.1016/S0733-8619(03)00101-4

Neurologic Clinics

Volume 22, Issue 1, February 2004, Pages 229-236

https://doi.org/10.1016/S0733-8619(03)00101-4 Get rights and content

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Radiographic and clinical correlations

More than a century after his initial research and publications, Chiari's classification scheme remains widely accepted. The neuroradiologic advance of MRI enables detection of Chiari malformations without the need for invasive procedures (Fig. 1). With routine use of this imaging modality, incidental cerebellar ectopia is observed in subjects who have varying and often poorly localizing symptoms, prompting numerous investigations dedicated to offering radiographic determination of these

Pathophysiology of tonsillar ectopia and headache

Chiari observed hindbrain deformities in patients who had hydrocephalus and considered these anomalies a result of congenital or chronic hydrocephalus, specifically not “acquired” or of late onset [4]. He questioned, however, if chronic hydrocephalus was sufficient to underlie elongation and the conical conformation of the tonsils as characterized in the type I anomaly. In Chiari's subsequent publication on the topic [2], he postulated an alternative mechanism of deficient bone growth with poor

Therapeutic considerations

As discussed previously, patients who have tonsillar ectopia may present with several headache types, including the common occipital headache, migraine, or tension type. Medications, such as nonsteroidal drugs, triptans, tricyclic antidepressants, acetazolamide, COX-2 inhibitors, butalbital, and opioids, are used in these patients with fair results [25]. Those who fail oral medications are sometimes referred for intramuscular injections of corticosteroids and local anesthetic agents in the

Summary

Tonsillar ectopia, encompassing slight descent of the cerebellar tonsils and Chiari I malformations, are disorders observed routinely in older children and adults and are believed to be an acquired form of the Chiari malformations. This entity is different from the other Chiari malformations in that hydrocephalus plays no role in its evolution. More likely it is a disorder of para-axial mesoderm, characterized by posterior fossa hypoplasia and content overcrowding, and not an embryologic

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Cited by (28)

Pituitary Adenoma Concomitant with Chiari I Malformation: Case Report and Literature Review
2019, World Neurosurgery
Citation Excerpt :
The only exceptions to this rule are the prolactinomas, for which medical management with dopamine agonists is the first line of treatment. By contrast, CIM is an uncommon finding, seen in around 0.56% to 3% of the general population,17,18 with an incidence of 3.3 to 8.2 per 100,000 individuals.19 It may produce medullar or upper cervical cord dysfunction during childhood and early adulthood, although some patients become symptomatic only until the fourth or fifth decade of life.4
Pituitary adenomas (PA) are usually benign neoplasms. Chiari I malformation (CIM) is an uncommon finding. Isolated cases associating functional PA and CIM have been reported. The concomitant presence of a nonfunctional PA and a CIM has not yet been described in the literature.
We present the case of a 35-year-old patient whose symptoms were compatible with CIM. Magnetic resonance imaging of the brain, with and without contrast material, confirmed the CIM and incidentally encountered a PA. The patient was treated initially for her PA through an endoscopic endonasal approach with complete tumor resection, confirming a nonfunctional PA. Posterior fossa decompression was successfully accomplished 3 months later. The patient has been followed up for 5 years and has been free of symptoms, with no recurrence of her PA. This represents the first reported case of a nonfunctional PA with a concomitant CIM.
This is the first reported case of a concomitant CIM and a nonfunctional PA. We discuss our successful management and conduct a systematic review of the literature to provide the most up-to-date guidance on managing these singular cases with concomitant pathologic conditions.
Chiropractic Care for Headaches and Dizziness of a 34-Year-Old Woman Previously Diagnosed With Arnold-Chiari Malformation Type 1
2014, Journal of Chiropractic Medicine
Citation Excerpt :
A diagnosis of ACM type 1 is often suspected following physical examination and history. Patients often complain of headaches in the posterior-occipital area, radiating to the eyes with a pressure-type pain made worse with coughing, sneezing, or yelling.1,10 Confirmation of ACM can only be made with imaging, most often made by cervical magnetic resonance imaging (MRI), which is the criterion standard; however, this is also seen on brain MRI, computed tomography, and cervical computed tomography.8
The purpose of this case study is to describe the chiropractic care of a patient with headaches and dizziness.
A 34-year-old woman with a history of headaches, dizziness, photophobia, and temporary loss of vision aggravated by postural positions while bending forward sought conservative care for her symptoms. She reported a prior diagnosis of Arnold-Chiari malformation (ACM) type 1 by magnetic resonance imaging in 2005 that revealed descending cerebellar tonsils measured at 5 mm with an impression of ACM type 1. A new magnetic resonance image taken in 2013 indicated the cerebellar tonsils measured at 3 mm and did not project through the plane of the foramen magnum. The diagnosis of ACM type 1 was no longer applicable; however, the signs and symptoms of ACM type 1 persisted.
She was treated using cervical chiropractic manipulation using diversified technique. The dizziness and headache were resolved after 3 visits. At her 3-month follow-up, she continued to be symptom-free.
A patient with headaches and dizziness and a previous diagnosis of ACM type 1 responded positively to chiropractic care.
Chiari Type I Malformation in a Pediatric Population
2009, Pediatric Neurology
Citation Excerpt :
This measurement is to some degree arbitrary, and does not include other anatomic factors that may determine if a patient develops symptoms or not. Whether individuals with 2-4 mm of tonsillar ectopia may also exhibit Chiari symptoms that respond to surgical intervention remains a matter of debate [17]. With the increasing availability of diagnostic magnetic resonance imaging, more asymptomatic patients are being identified [4,5,18].
The natural history of Chiari I malformation in children remains unclear. A population-based retrospective cohort study was therefore conducted. Radiology reports from all head and spine magnetic resonance imaging scans (n = 5248) performed among 741,815 children under age 20 within Kaiser Northern California, 1997-1998, were searched for Chiari I. Medical records and imaging studies were reviewed to determine clinical and radiographic predictors of significant neurologic symptoms, defined as moderate to severe headache, neck pain, vertigo, or ataxia. The 51 patients identified with Chiari I represented 1% of the children who had head or spine magnetic resonance imaging scans performed during the study period. Headache (55%) and neck pain (12%) were the most common symptoms. Syringomyelia was present in 6 patients (12%) at initial diagnosis; no new syrinxes developed during follow-up. Older age at time of diagnosis was associated with increased risk of headache (odds ratio OR = 1.3, 95% confidence interval CI = 1.1-1.5) and significant neurologic symptoms (OR = 1.2, 95% CI = 1.04-1.4). Chiari I, an underrecognized cause of headaches in children, is also frequently discovered incidentally in children without symptoms. Larger and longer-term studies are needed to determine the prognosis and optimal treatment of pediatric Chiari I.
Diagnostic Testing for Migraine and Other Primary Headaches
2009, Neurologic Clinics
Citation Excerpt :
Chiari I malformation typically is a congenital malformation of cerebellar tonsillar herniation at least 5 cm below the foramen magnum. The headache attributed to Chiari I malformation is occipital or nuchal-occipital with occasional radiation unilaterally to frontotemporal or shoulder regions and sometimes generalized.38 The pain may be dull, aching, or throbbing and may last less than 5 minutes to several hours to days.
Most primary headaches can be diagnosed using the history and examination. Judicious use of neuroimaging and other testing, however, is indicated to distinguish primary headaches from the many secondary causes that may share similar features. This article evaluates the reasons for diagnostic testing and the use of neuroimaging, electroencephalography, lumbar puncture, and blood testing. The use of diagnostic testing in adults and children who have headaches and a normal neurologic examination, migraine, trigeminal autonomic cephalalgias, hemicrania continua, and new daily persistent headache are reviewed.
Intracranial incidental findings on brain MR images in a pediatric neurology practice: A retrospective study
2008, Journal of the Neurological Sciences
Previous studies have addressed the prevalence of incidental findings largely in healthy adult and pediatric populations. Our study aims to elucidate the prevalence of incidental findings in a pediatric neurology practice.
We reviewed the charts of 1618 patients seen at a pediatric neurology practice at a tertiary care center from September 2003 to December 2005 for clinical data and incidental intracranial findings on brain magnetic resonance imaging reports. Incidental findings were divided into two categories: normal or abnormal variants. Clinical and demographic data were assessed for associations with incidental findings.
From 1618 charts reviewed, only 666 patients (41% of all patients) had brain MRIs ordered. One-hundred and seventy-one (171) patients (25.7% of all patients; 95% CI: 22.6, 29.0) had incidental findings. Of these, 113 (17.0%; 95% CI: 14.1, 19.8) were classified as normal-variants and 58 (8.7%; 95% CI: 6.6, 10.9) were classified as abnormal. The nature of incidental findings was not related to age group, sex or clinical diagnosis (p = 0.29, p = 0.31 and p = 0.69 respectively). Two patients (0.3%; 95% CI: ∼ 0.0, 0.7) required neurosurgical referral.
We report a high prevalence of and a low rate of referrals for incidental findings in comparison to previous studies. The present study may help guide management decisions and discussions with patients and families. Future studies should attempt to address issues of associations between primary or secondary diagnoses and intracranial incidental findings in a controlled, prospective fashion.
Prevalence of Chiari malformation type 1 is increased in pseudohypoparathyroidism type 1A and associated with aberrant bone development
2023, PLoS ONE

View all citing articles on Scopus

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Review articleTonsillar ectopia and headaches

Section snippets

Radiographic and clinical correlations

Pathophysiology of tonsillar ectopia and headache

Therapeutic considerations

Summary

First page preview

Magn Reson Imaging

Clin Radiol

Oral Surg Oral Med Pathol Oral Radiol Endod

J Manipulative Physiol Ther

Ueber Veränderugen des Kleinhirns infolge von Hydrocephalie des Grosshirns

Dtsch Med Wochenschr

Uber Veränderugen des Kleinhirns, des Pons und der Medulla Oblongata infolge von Congenitaler Hydrocephalie des Grosshirns

Denkschr der Kais Akad Wiss Wien mathnaturw

Concerning alterations in the cerebellum resulting from cerebral hydrocephalus

Pediatr Neurosci

History of neurology: seminal citation. Arnold-Chiari Malformation

Arch Neurol

Position of cerebellar tonsils in the normal population and in patients with Chiari malformation: a quantitative approach with MR imaging

J Comput Assist Tomogr

Chiari I Malformation: from Dr Chiari to MR imaging

Radiology

Significance of cerebellar tonsillar position on MR

AJNR Am J Neuroradiol

Chiari I malformations: clinical and radiological reappraisal

Radiology

Chiari I malformation redefined: clinical and radiographic findings for 364 symptomatic patients

Neurosurgery

Symptomatic Chiari malformation in adults: a new classification based on magnetic resonance imaging with clinical and prognostic significance

Neurosurgery

Apparent cerebellar ectopia: a reappraisal using volumetric MRI

Neuroradiology

Myelographic examination of the posterior fossa with positive contrast medium

Radiology

Comparison of presenting symptoms and neurological signs in incidental tonsillar ectopia

Neurology

Symptomatic tonsillar ectopia

J Neurol Neurosurg Psychiatry

Review article
Tonsillar ectopia and headaches