Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0018681 (headache)
 56,091 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

HTLV-I is associated with a broad spectrum of manifestations, including tropical spastic paraparesis and adult T-cell leukemia/lymphoma. Arnold Chiari syndrome is a condition characterized by herniation of the cerebellar tonsils through the foramen magnum. This condition should be suspected in all patients with headache and impaired motor coordination. Syringomyelia is a developmental anomaly that leads to the formation of an intramedullary cavity. Its clinical presentation is classically characterized by syringomyelic dissociation of sensation, with suspended distribution in the proximal portion of the trunk and upper limbs and preservation in other regions. We report here a case of association of the three diseases, which is rare in clinical practice, illustrating the difficulty in the diagnosis and therapeutic management of these conditions.
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PMID:Association of HTLV-I with Arnold Chiari syndrome and syringomyelia. 1928 45

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.
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PMID:Chiari type I malformation in a pediatric population. 1943 79

To restore the cerebrospinal fluid (CSF) flow at the craniocervical junction in Chiari I malformation (CM-I), most surgeons practice a suboccipital craniectomy with duraplasty. To reduce the risk of CSF leak, a dura splitting decompression is created removing only the dural outer layer. We report on a series of 11 patients with CM-I (five with syringomyelia) operated on between 2000 and 2007 using this technique. Neurological examination and cerebro-spinal MRI scan were performed before and after surgery. Symptoms improved completely in six patients. Headaches and cervicalgias disappeared for all patients. Dizziness and paresthesia in the upper limb remained unchanged for three and two patients, respectively. We observed no complications such as CSF leak, meningocele, or meningitis. Postoperative MRI scan showed a significant craniocervical decompression in ten patients. Four patients had a new cisterna magna. Two syringomyelias completely disappeared, two decreased, and one was stable. Dural splitting can be practiced to treat CM-I. Clinical results are similar to the other techniques with less complications. Radiological findings show satisfying posterior fossa decompression.
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PMID:Dura splitting decompression in Chiari type 1 malformation: clinical experience and radiological findings. 1964 15

CSF flow has been shown to exhibit complex patterns in MR images in both healthy subjects and in patients with Chiari I. Abnormal CSF flow oscillations, according to prevailing opinion, cause syringomyelia and other clinical manifestations that affect some patients with the Chiari I malformation. For this article, we reviewed the literature on PC MR of CSF flow, collected the published CFD studies relevant to CSF flow, and performed flow simulations. PC MR creates cine and still images of CSF flow and measurements of flow velocities. CFD, a technique used to compute flow and pressure in liquid systems, simulates the CSF flow patterns that occur in a specific geometry or anatomy of the SAS and a specific volume of flow. Published PC MR studies show greater peak CSF velocities and more complex flow patterns in patients with Chiari I than in healthy subjects, with synchronous bidirectional flow one of the characteristic markers of pathologic flow. In mathematic models of the SAS created from high-resolution MR images, CFD displays complex CSF flow patterns similar to those shown in PC MR in patients. CFD shows that the pressure and flow patterns vary from level to level in the upper spinal canal and differ between patients with Chiari and healthy volunteers. In models in which elasticity and motion are incorporated, CFD displays CSF pressure waves in the SAS. PC MR and CFD studies to date demonstrate significant alterations of CSF flow and pressure patterns in patients with Chiari I. CSF flow has nonlaminar complex spatial and temporal variations and associated pressure waves and pressure gradients. Additional simulations of CSF flow supplemented by PC MR will lead to better measures for distinguishing pathologic flow abnormalities that cause syringomyelia, headaches, and other clinical manifestations in Chiari I malformations.
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PMID:Characterization of cyclic CSF flow in the foramen magnum and upper cervical spinal canal with MR flow imaging and computational fluid dynamics. 2022 87

The objective of this study was to analyze the association of cerebellar tonsillar descent and syringomyelia in patients with posterior fossa arachnoid cysts. We reviewed the medical records of ten patients (mean, age 33; range, 24-49 years) diagnosed with posterior fossa arachnoid cyst and tonsillar descent. Symptoms evolved over a mean of 12 months (range, 6 months to 3 years). Syringomyelia was present in six cases. Six patients underwent a suboccipital craniectomy, three cases underwent an additional C1 laminectomy, and a further case had a limited craniectomy and tonsillar reduction. Three patients were also treated for hydrocephalus: one with a ventriculoperitoneal shunt and two with endoscopic third ventriculostomy. Two patients had conservative treatment. The posterior fossa arachnoid cysts were located at the vermis-cisterna magna (n = 4), the cerebellar hemispheres (n = 2), the cerebellopontine angle (n = 3), and the quadrigeminal cistern (n = 1). A patient with achondroplasia showed features of platybasia. Associated malformations included craniofacial dysmorphism in a patient diagnosed of trichorhinophalangeal syndrome and a case with a primary temporal arachnoid cyst. After a mean follow-up of 2 years (range, 3 months to 5 years), four patients showed resolution of their neurological symptoms, and two exhibited persisting ocular findings. Headaches and nuchalgia improved in four cases and persisted in four. Syringomyelia was resolved in four patients and improved in two. Patients harboring a posterior fossa arachnoid cyst may evolve with acquired Chiari malformation and syringomyelia. Initial management should be directed to decompressing the foramen magnum and should include the resection of the arachnoid cyst's walls. A wait-and-see attitude can be implemented in selected cases. In our experience, hydrocephalus should be properly addressed before treating the arachnoid cyst.
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PMID:Posterior fossa arachnoid cysts and cerebellar tonsillar descent: short review. 2048 Mar 82

Parry-Romberg syndrome is characterized by progressive unilateral facial atrophy affecting subcutaneous tissue, cartilage and bone structures. Headache attacks and epilepsy are commonly associated with this syndrome but the underlying pathophysiology is still unknown. A case of a 12-year-old boy with Parry-Romberg syndrome and syringomyelia suffering from severe headache attacks and epileptic seizures is reported herein. Headache attacks were associated with bilateral autonomic symptoms and hyperventilation and were usually followed by complex partial and sometimes by secondary generalized tonic seizures. Detailed neuroimaging examinations were performed (magnetic resonance imaging [MRI] of the head, orbits, and spinal cord, MR angiography, and MR spectroscopy of the cerebellum). The EEG pattern revealed localized discharges contralaterally to the affected side. Antiepileptic treatment with carbamazepine was instituted with minimal effect. Modification of treatment (replacement with oxcarbazepine) was successful. In the reported patient interesting correlation of headache attacks, autonomic symptoms and epileptic seizures was observed. Additionally we believe it is the first report of coincident syringomyelia and Parry-Romberg syndrome.
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PMID:Diagnostic difficulties of paroxysmal symptoms in a boy with Parry-Romberg syndrome. 2062 66

Chiari type I malformation is found in 1 out of 20 magnetic resonance imaging (MRI) studies. Isolated tonsillar herniation is of limited utility and should be considered within the clinical context because these patients can be asymptomatic. Cine MRI showing compression of the cerebrospinal fluid (CSF) spaces in the foramen magnum area is a crucial technique for making treatment decisions. Congenital malformation is thought to be due to a volumetric small posterior fossa. The most common symptom in these patients is cough headache. Posterior fossa reconstruction is mandatory in patients with progressive symptoms/signs, hydrocephalus, or syringomyelia, but not in patients who are asymptomatic or those with stable and tolerable symptoms. Acquired tonsillar descent can be secondary to a variety of disorders conditioning disproportion between the volume of the cranial cavity and that of the intracranial contents, or to CSF hypovolemia, which is the most common cause for acquired herniation. CSF hypovolemia can be spontaneous or secondary to CSF removal. Treatment of acquired tonsillar herniation depends on the responsible etiology.
Curr Pain Headache Rep 2011 Jun
PMID:What differences exist in the appropriate treatment of congenital versus acquired adult Chiari type I malformation? 2132 65

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 management of intradiploic CSF collection is controversial. Although it is a benign lesion, even then delay in diagnosis and treatment may lead to significant morbidity. The authors report a very rare case of giant posttraumatic intradiploic pseudomeningocele involving the occipital bone, occipital condyles, and clivus. The pathogenesis and management of intradiploic CSF collection are discussed. This 16-year-old boy presented with a history of enlarging swelling in the suboccipital region associated with headache, lower cranial nerve palsy, and features of high cervical compressive myelopathy. Investigations revealed a giant intradiploic lesion involving the occipital bone, condyles, and clivus associated with secondary basilar invagination, hydrocephalus, and syringomyelia. Intrathecal contrast administration did not reveal communication of intradiploic space with the subarachnoid space. A large occipital craniotomy was performed. A linear fracture and dural defect in the midline was identified, which was closed with fascial graft after removing the inner table of the skull. Cranioplasty was performed using the expanded calvarial bone. Ventriculoperitoneal shunt insertion was performed for hydrocephalus, and the patient improved remarkably. Posttraumatic intradiploic CSF collection, although a benign condition, may present with severe complications if treatment is delayed. Early diagnosis and treatment is essential. The authors suggest that this condition should be treated early, as for growing skull fractures.
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PMID:Giant intradiploic pseudomeningocele of occipital bone. 2220 26

Subarachnoid blood has been reported as a cause of chronic spinal arachnoiditis. Although syringomyelia has been thought to be caused by spinal arachnoiditis, reports of syringomyelia following aneurysmal subarachnoid hemorrhage (SAH) are very rare. We describe two patients with syringomyelia associated with chronic spinal arachnoiditis following SAH. From January 2001 to December 2010, 198 patients with aneurysmal SAH were treated at Kinki University School of Medicine. Two of the 198 patients had syringomyelia following aneurysmal SAH; thus the rate of syringomyelia associated with aneurysmal SAH was 1.0%. Patient 1 was a 54-year-old woman who presented with back pain, back numbness and gait disturbance 20 months after SAH. Her MRI revealed syringomyelia of the spinal cord from C2 to T10. She underwent shunting of the syrinx to the subarachnoid space. Patient 2 was a 49-year-old man, who was admitted to the hospital with headache, diplopia, hoarseness, dysphagia and ataxia five months after SAH. MRI revealed syringomyelia from the medulla oblongata to C6, and an enlargement of the lateral and fourth ventricles. After foramen magnum decompression and C1 laminectomy, a fourth ventricle-subarachnoid shunt was placed by insertion of a catheter. Spinal arachnoiditis and spinal syringomyelia are rare but important chronic complications after SAH.
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PMID:Spinal syringomyelia following subarachnoid hemorrhage. 2228 78


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