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Query: UMLS:C0018681 (headache)
 56,091 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Two Williams syndrome patients are presented who had neurologic symptoms secondary to Chiari malformation type I. Both patients had many of the well-known medical problems found in Williams syndrome. In addition, Patient 1 developed headache, diplopia, and tinnitus at 26 years of age. Neurologic examination revealed intermittent nystagmus and brisk reflexes. Magnetic resonance imaging demonstrated Chiari malformation type I; neurologic symptoms abated following surgery. Patient 2 had a normal neurologic examination at 2 years of age except for hyperreflexia and tight heel cords. At age 10 years, she had generalized contractures, decreased strength and wasting of hand musculature, and hyperreflexia. Magnetic resonance imaging documented Chiari malformation type I. Both patients have significant dysphagia and fusion of cervical spine segments noted on radiography. Morphometric analyses of intracranial contents based on midsagittal magnetic resonance images were performed. This analysis suggests that, compared to age-matched controls, the posterior fossa size is selectively diminished in Williams syndrome, whereas the cerebellum is normal in size. This "mismatch" between the size of the posterior fossa bony compartment and its neural contents may place Williams syndrome patients at high risk for developing Chiari malformation type I.
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PMID:Association of Chiari I malformation and Williams syndrome. 774 69

Adult Arnold-Chiari malformation, also known as Chiari malformation type I, typically occurs in women during early adulthood and can be a cause of unexplained headaches, as well as associated syringomyelocele. In this unique case report, a 32-year-old, postpartum woman with posturally induced headache from Chiari malformation type I had symptoms occur for the first time during pregnancy. Magnetic resonance imaging of the brain confirmed the diagnosis and is the neuroimaging study of choice in such cases. Neurosurgical decompression, when performed early, is highly successful in symptomatic individuals.
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PMID:Adult Arnold-Chiari malformation: a postpartum case presentation. 855 51

A relatively common group of developmental anomalies of the posterior fossa and its contents make up the Chiari malformations. The most common of these anomalies are the Chiari malformations type I and type II. Many patients with these defects are asymptomatic, but many of them suffer from headaches. Although the prevalence of common primary headache disorders in patients with Chiari malformations is similar to that of the general population, a particular type of occipital headaches occurs more frequently in patients with a Chiari malformation. In the two patients described in the illustrative case presentations, the major complaint was headache. Both had Chiari malformation type I diagnosed. Chiari malformation should be considered in the differential diagnosis of patients who complain of exertional headache. Exertional headaches or worsening of pain during Valsalva's maneuver should prompt clinical suspicion of an underlying intracranial lesion and warrants further evaluation by appropriate neuroimaging studies.
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PMID:Headache in Chiari malformation: a distinct clinical entity? 1021 9

Benign exertional headache is coded as a separate entity within the International Headache Society's classification system, but the pathophysiological mechanisms underlying this clinical headache subtype are unknown and possibly are similar to those generating migraine. Coexistence of migraine and benign exertional headache in the same patient is not unusual, and antimigraine pharmacologic treatments are often effective in both headache types. Regardless, optimal management mandates that the clinician exclude any intracranial or systemic disease that could mimic "primary" exertional headache. The same holds for primary headaches induced by coughing or sneezing; congenital malformations or neoplasms, particularly within the posterior fossa, are not rare in these patients. The neurologic examination may not be sufficiently sensitive to detect the offending lesion. We describe a patient with migraine without aura and exertional secondary headache due to Chiari malformation type I whose headaches responded to treatment with propranolol and indomethacin.
Headache 2003 Apr
PMID:Chiari-associated exertional, cough, and sneeze headache responsive to medical therapy. 1265 13

The Chiari type I malformation (CM1) is characterized by herniation of cerebellar tonsils to at least 3-5 mm below the plane of foramen magnum and can present with a wide variety of clinical symptoms, frequently including occipital headaches, secondary to bulbar and/or medullary distress. Rarely, syncopal episodes have also been described and attributed to either compression of the midbrain ascending reticular system, or vascular compromise (vertebrobasilar artery compression, hypotension). We report the first case of a CM1 patient with frequently recurring syncope due to postural orthostatic tachycardia syndrome (POTS), a form of orthostatic intolerance, whose symptoms resolved completely after surgical intervention. It is important to stress that it is not clear whether the described association of POTS and CM1 in our patient is a fortuitous finding in an isolated case or a reflection of a more systematic association between the two pathologies.
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PMID:Orthostatic intolerance and syncope associated with Chiari type I malformation. 1596 23

This report presents three families with Chiari malformation type I that became symptomatic during childhood: a mother and son; a set of monozygotic twins; and two half-siblings and their two maternal cousins. These children presented with various symptoms, including headache, stiff neck, and swallowing difficulty. A review of the relevant literature is presented, with an emphasis on familial examples and proposed inheritance. Less common presentations of Chiari malformation type I are discussed, as well as the possible pathogenesis of Chiari malformation type I and associated syringomyelia.
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PMID:Chiari in the family: inheritance of the Chiari I malformation. 1676 29

This chapter deals with non-vascular intracranial disorders resulting in headache. Headache attributed to high or low cerebrospinal fluid pressure is separated into headache attributed to idiopathic intracranial hypertension (IIH), headache attributed to intracranial hypertension secondary to metabolic, toxic, or hormonal causes, headache attributed to intracranial hypertension secondary to hydrocephalus, post-dural puncture headache, cerebrospinal fluid (CSF) fistula headache, headache attributed to spontaneous (or idiopathic) low CSF pressure. Headache attributed to non-infectious inflammatory disease can be caused by neurosarcoidosis, aseptic (non-infectious) meningitis or lymphocytic hypophysitis. Headache attributed to intracranial neoplasm can be caused by increased intracranial pressure or hydrocephalus caused by neoplasm or attributed directly to neoplasm or carcinomatous meningitis. Other causes of headache include hypothalamic or pituitary hyper- or hyposecretion and intrathecal injection. Headache attributed to epileptic seizure is separated into hemicrania epileptica and post-seizure headache. Finally headache attributed to Chiari malformation type I (CM1) and the syndrome of transient headache and neurological deficits with cerebrospinal fluid lymphocytosis (HaNDL) are described.
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PMID:Headache attributed to non-vascular intracranial disorder. 2081 56

Headache attributable to nonvascular intracranial disorder is a basket of multiple, partly complex, and very diverse idiopathic or secondary disorders. By definition, the headache has to occur in a close temporal relationship to the intracranial disorder. Some of these headache disorders are caused by high or low cerebrospinal fluid pressure; noninfectious inflammatory diseases such as neurosarcoidosis, aseptic (noninfectious) meningitis, and lymphocytic hypophysitis; or intracranial neoplasm. Other nonvascular headaches, including hemicrania epileptica and postseizure headache, Chiari malformation type I, and the syndrome of transient headache and neurological deficits with cerebrospinal fluid lymphocytosis, are attributed to hypothalamic or pituitary hyper- or hyposecretion, intrathecal injection, or epileptic seizures. The clinical presentation of all these disorders can be diverse and often mimics the characteristics of primary headaches, which may delay the diagnosis.
Curr Pain Headache Rep 2011 Aug
PMID:Headache attributable to nonvascular intracranial disorders. 2145 38

Chiari malformation type I and Poland's syndrome are two rare diseases and their simultaneous presentation had not been previously described in the literature. We report the case of a 27 year old male with history of Poland's syndrome, who referred headache and motor impairment of the intrinsic muscles of the left hand. In a cervical spine MR a Chiari I malformation with syringomyelia from C1 to T2 was found, which was treated by foramen magnum decompression, dural plasty and removal of the posterior arch of the atlas. A discussion of the embryological mechanisms that might be involved in the coexistence of these two entities is presented, emphasizing the role of para-axial mesoderm.
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PMID:[Chiari type I malformation in a patient with Poland's syndrome]. 2216 90

The most typical symptom of Chiari malformation type I in children is headache. The authors describe a 14-year-old girl who presented with a 3-year history of gait decline and no headache, which is very unusual. After surgery to correct the Chiari I malformation, the patient's gait improved; however, she went on to develop decreased hand use with joint deformities. She was diagnosed with a probable connective tissue disorder. Patients with connective tissue disorders are at increased risk for developing Chiari I malformation. The authors discuss the possible reasons for the unusual presentation of the Chiari I malformation and possible mechanisms. The unusual presentation of Chiari I delayed this young patient's diagnosis and treatment.
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PMID:An Unusual Presentation of Chiari I Malformation. 2303 77


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