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

CSF pressure recordings have been taken from the lumbar region and the cisterna magna of 16 patients during coughing in the sitting position. Isolated coughs of low amplitude have been studied. The lumbar pressure waves occur sooner and lower. Thus there is a phase during which the lumbar pressure exceeds the cisternal, followed by one in which the cisternal exceeds the lumbar. These phenomena may be conveniently displayed on a differential trace. The phase during which the cisternal pressure exceeds the lumbar may be protracted. It is suggested that Froin's syndrome, central subarachnoid pouches and syringobulbia may be associated with upward pressure waves. Cough headache, the filling stages of communicating syringomyelia and tonsillar herniation may be associated with valve-like blockage at the foramen magnum which produces craniospinal pressure dissociation by interfering with downward or rebound pulsation. Decompensation of hydrocephalus after birth may be related to pulsation in association with crying; also after removal of a meningocele sac decompensation may be related to the effects of similar pulsation modified by changes in capacitance following operation. The cord destruction of syringomyelia, and the mechanisms which fill spinal subarachnoid cysts may be related to pressure waves directed both upwards and downwards. The merits and limitations of cough impulse as a clinical test for spinal blockage are discussed, and the suggestion is made that after further evaluation they may provide a more sensitive indication of spinal blockage than Queckenstedt's test.
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PMID:Cerebrospinal fluid pressure changes in response to coughing. 99 Sep

Syringobulbia is a term which has been clinically applied to brain stem symptoms or signs in patients with syringomyelia. Syringobulbia clefts are found on investigation or at necropsy caused by cutting outwards of the CSF under pressure from the fourth ventricle into the medulla. These should be differentiated from the ascending syringobulbia which may occur from upward impulsive fluid movements in a previously established syringomyelia. Clinical analysis of 54 patients suggests that bulbar features are most often found with neither of the above mechanisms but are due to the effects of pressure differences acting downward upon the hind-brain with consequent distortion of the cerebellum and brainstem, traction on cranial nerves or indentation of the brain-stem by vascular loops. The commonest symptoms in the 54 patients were headache (35), vertigo (27), dysphonia or dysarthria (21), trigeminal paraesthesiae (27), dysphagia (24), diplopia (16), tinnitus (11), palatal palsy (11) and hypoglossal involvement (11). Careful attention to hydrocephalus is advisable before craniovertebral surgery, but the decompression of the hindbrain and the correction of craniospinal pressure dissociation remains the mainstay of surgical treatment. The results of careful surgery are good, 45 of the 54 cases reported improvement. Most of the reported deterioration occurred in a few patients who did conspicuously badly.
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PMID:Syringobulbia: a surgical appraisal. 147 91

A case of solitary syringobulbia without syringomyelia diagnosed by MRI was reported. A 63-year-old female, who had a history of progressive gait disturbance for 9 months, was admitted to our department because of severe headache. Neurological examination revealed that the patient was suffering from right pyramidal sign as well as left deep sensory disturbance without apparent impairment of cranial nerves or nystagmus. The somatosensory evoked potential stimulated by the left median nerve showed delay in latency and low amplitude, however, the auditory brainstem response showed normal records bilaterally. A syringobulbia associated with platybasia, basilar impression, obstructive hydrocephalus and Arnold-Chiari malformation was diagnosed by the neuroradiological examinations including plain X-ray films of the skull, CT scan and MRI of the brain. Subsequent to the decompression around the cisterna magna by suboccipital craniectomy and laminectomy of the atlas, the opening of the Foramen Monro which was obstructed by a thick membrane was carried out, and prevention of communication between the fourth ventricle and the syrinx was accomplished by using a piece of muscle. This operative procedure was thought to be compatible with Gardner's operation for syringomyelia. The patient showed gradual improvement of gait disturbance as well as headache 3 months after surgery, and this neurological improvement was proved by decrease in the size of the known syringobulbia on MRI, and by normalized somatosensory evoked potential stimulated by the median nerve. Pathophysiology of the solitary syringobulbia with relatively acute onset of the neurological symptoms was assumed to have taken place in the following manner.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[A case of solitary syringobulbia]. 200 Jan 59

Herniation of the hindbrain occurs when the lowest parts of the cerebellum and sometimes part of the medulla are moved downwards through the foramen magnum, a pressure difference acting across the foramen magnum moulding the tissues into a plug. It is suggested that the clinical course in both adults and babies with spina bifida may be explained by the hindbrain hernia acting as a valve.The term 'Chiari Type I deformity' is commonly used for an abnormality in which the tonsils and lowermost parts of the cerebellar hemispheres are prolapsed through a normal foramen magnum. Acute herniation may occur as a result of space-occupying lesions. Chronic herniation may be morphologically identical although it tends to be more severe. Sometimes it will produce few symptoms which often may be delayed so that the original causative lesion may not be apparent. Causes include bone softening, tumour, or previous meningitis. Birth injury is probably the commonest cause of the deformity, which presents clinically in adults.In infants with severe forms of spina bifida a hindbrain herniation is present. This abnormality may be called 'Chiari Type II deformity' or Arnold-Chiari deformity and is an intra-uterine abnormality in which the fourth ventricle and medulla are grotesquely herniated before they are properly developed and the foramen magnum is enlarged.The commonest clinical presentation of Chiari Type I deformity is syringomyelia, which is usually not diagnosed until adult life. Other presentations include syringobulbia, headache, oscillopsia, attacks of giddiness, lower cranial nerve palsies, and ataxia. Particularly characteristic are cough headache and cough syncope. Syringomyelia and syringobulbia in particular may be irreversible by the time they are diagnosed. Nevertheless, surgical decompression may be successful in relieving symptoms of headache, cough syncope, and long-tract compression; most cases of syringomyelia show some improvement and in others progression of the disease is arrested. Operative techniques for hindbrain herniation are discussed.Chiari Type II deformity is probably responsible for the progression of hydrocephalus after birth in the majority of babies with spina bifida. Measurement of pressure in the cerebrospinal fluid above and below the foramen magnum shows that intermittent pressure difference is commonly present at times of neurological deterioration. Surgical decompression of the hernia in adults allows correction of the valvular effect, which may be monitored by pressure measurements. In babies the associated hydrocephalus is usually so gross that it requires separate treatment, but pressure monitoring may be of value in assessing the state of the disease.
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PMID:Chronic herniation of the hindbrain. 701 51

Syringobulbia is an uncommon lesion of the central nervous system. It is defined as a pathological cavitation of the brain stem. The most common symptoms are headache, vertigo, dysphonia or dysarthria, trigeminal paraesthesia, dysphagia, diplopia, tinnitus, palatal palsy. Syringobulbia occurs with atlantoaxial congenital abnormalities (Chiari malformation), infection, tumours, and other causes. The idiopathic syringobulbia is however a rare finding. Early surgical treatment is the treatment of choice. We report on a 58-year old female patient with idiopathic syringobulbia. She complained of occipital headaches and vertigo. On examination she had horizontal nystagmus and diplopia. Occipital headaches and vertigo were improved after operation. We review the literature on syringobulbia, and discuss the clinical features of this uncommon condition.
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PMID:[Isolated idiopathic syringobulbia: case report and summary of the literature]. 877 68

Osteogenesis imperfecta (OI) is a heritable disorder of bone development caused by defective collagen synthesis. Basilar invagination is an uncommon but devastating complication of this disease. The authors present a comprehensive strategy for management of craniovertebral anomalies associated with OI and related osteochondrodysplasias. Twenty-five patients with congenital osteochondrodysplasias (18 OI, four Hajdu-Cheney syndrome, and three spondyloepiphyseal dysplasia) and basilar invagination were evaluated between 1985 and 1995. The male/female ratio in this cohort was 1:1. The mean age at presentation was 11.9 years (range 13 months-20 years). Fourteen patients (56%) presented during adolescence (11-15 years of age). Symptoms and signs included headache (76%), lower cranial nerve dysfunction (68%), hyperreflexia (56%), quadriparesis (48%), ataxia (32%), nystagmus (28%), and scoliosis (20%). Four patients (16%) were asymptomatic. Seven (28%) had undergone previous posterior fossa decompression; one had also undergone ventral decompression. Imaging findings included basilar invagination (100%), ventral brainstem compression (84%), hydrocephalus (32%), hindbrain herniation (28%), and syringomyelia/syringobulbia (16%). Patients with hydrocephalus underwent ventricular shunt placement. Reducible basilar invagination (40%) was treated with posterior fossa decompression and occipitocervical fusion. Those with irreducible ventral compression (60%) underwent transoral-transpalatopharyngeal decompression followed by occipitocervical fusion. All patients improved initially. However, basilar invagination progressed radiographically in 80% (symptomatic in 24%) despite successful fusion. Prolonged external orthotic immobilization with the modified Minerva brace afforded symptomatic improvement and arrested progression of the deformity. The mean follow-up period was 5.9 years (range 1.1-10.5 years). Ventral brainstem compression in OI should be treated with ventral decompression, followed by occipitocervical fusion with contoured loop instrumentation to prevent further squamooccipital infolding. Despite fusion, however, basilar invagination tends to progress. Prolonged immobilization (particularly during adolescence) may stabilize symptoms and halt further invagination. This study represents the largest series to date addressing craniovertebral anomalies in OI and related congenital bone softening disorders.
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PMID:Basilar invagination in osteogenesis imperfecta and related osteochondrodysplasias: medical and surgical management. 917 Nov 73

This is a case report of a male patient who presented with orofacial pain for a year as the only manifestation of syringobulbia-syringomyelia associated with Arnold-Chiari malformation. This article places emphasis on the clinical presentation and possible differential diagnoses. The pain was continuous and affected the left side of the face. It was exacerbated by coughing and physical effort, possibly as a consequence of an increase in intracranial pressure. Paroxysmal pain crises developed over this background of continuous pain, compatible with neurogenic trigeminal pain of the left second branch, together with pain episodes similar to cluster headache on the same side. The symptoms were resolved following neurosurgical management with amplification of the foramen magnum.
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PMID:Orofacial pain as the sole manifestation of syringobulbia-syringomyelia associated with Arnold-Chiari malformation. 1144 28

OBJECTIVE Syringobulbia (SB) is a rare entity, with few cases associated with Chiari malformation type I (CM-I) in the pediatric population. The authors reviewed all pediatric cases of CM-I-associated SB managed at their institution in order to better understand the presentation, treatment, and surgical outcomes of this condition. METHODS A prospectively maintained institutional database of craniovertebral junction abnormalities was analyzed to identify all cases of CM-I and SB from the MRI era (i.e., after 1984). The authors recorded presenting symptoms, physical examination findings, radiological findings, surgical treatment strategy, intraoperative findings, and outcomes. SB cases associated with tumors, infections, or type II Chiari malformations were excluded. RESULTS The authors identified 326 pediatric patients with CM-I who were surgically treated. SB was identified in 13 (4%) of these 326 patients. Headache and neck pain were noted in all 13 cases. Cranial nerve abnormalities were common: vagus and glossopharyngeal nerve dysfunction was the most frequent observation. Other cranial nerves affected included the trigeminal, abducens, and hypoglossal nerves. Several patients exhibited multiple cranial nerve palsies at presentation. Central sleep apnea was present in 6 patients. Syringomyelia (SM) was present in all 13 patients. SB involved the medulla in all cases, and extended rostrally into the pons and midbrain in 2 patients; in 1 of these 2 cases the cavity extended further rostrally to the cerebrum (syringocephaly). SB communicated with the fourth ventricle in 7 of the 13 cases. All 13 patients were treated with posterior fossa decompression with intradural exploration to ensure CSF egress out of the fourth ventricle and through the foramen magnum. The foramen of Magendie was found to be occluded by an arachnoid veil in 9 cases. Follow-up evaluation revealed that SB improved before SM. Cranial nerve palsies regressed in 11 of the 13 patients, and SB improved in all 13. CONCLUSIONS The incidence of SB in our surgical series of pediatric patients with CM-I was 4%, and all of these patients had accompanying SM. The SB cavity involved the medulla in all cases and was found to communicate with the fourth ventricle in 54% of cases. Posterior fossa decompression with intradural exploration and duraplasty is an effective treatment for these patients.
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PMID:Syringobulbia in pediatric patients with Chiari malformation type I. 2970 58

This study aimed to summarize the clinical features, diagnosis, and treatment of Chiari malformation type I- (CM-1-) associated syringobulbia. We performed a literature review of CM-1-associated syringobulbia in PubMed, Ovid MEDLINE, and Web of Science databases. Our concerns were the clinical features, radiologic presentations, treatment therapies, and prognoses of CM-1-associated syringobulbia. This review identified 23 articles with 53 cases. Symptoms included headache, neck pain, cranial nerve palsy, limb weakness/dysesthesia, Horner syndrome, ataxia, and respiratory disorders. The most frequently involved area was the medulla. Most of the patients also had syringomyelia. Surgical procedures performed included posterior fossa decompression, foramen magnum decompression, cervical laminectomy, duraplasty, and syringobulbic cavity shunt. Most patients experienced symptom alleviation or resolution postoperatively. A syringobulbic cavity shunt provided good results in refractory cases. Physicians should be aware of the possibility of syringobulbia in CM-1 patients, especially those with symptoms of sudden-onset brain-stem involvement. The diagnosis relies on the disorder's specific symptomatology and magnetic resonance imaging. Our review suggests that the initial therapy should be posterior fossa decomposition with or without duraplasty. In refractory cases, additional syringobulbic cavity shunt is the preferred option.
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PMID:Syringobulbia in Patients with Chiari Malformation Type I: A Systematic Review. 3101 90

Chiari 1.5 malformation (C1.5) is a rare condition characterized by caudal descent of the brain stem, together with descent of the cerebellar tonsils characteristic of Chiari I malformation (CIM). Syringobulbia (SB) is rarely associated with CIM and to date has not been reported in a patient with C1.5. An adolescent female presented with Valsalva-induced headaches and a left abducens nerve palsy. Imaging revealed C1.5 and a huge syringomyelia extending cranially into the herniated medulla oblongata as syringobulbia. Simultaneous cases of C1.5 and syringomyelia (SM) have been described, but, to our knowledge, this is the first reported case of simultaneous C1.5 and SB. As SB can have life altering consequences, the clinician should keep this possibility in their differential diagnosis.
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PMID:Case of simultaneous Chiari 1.5 malformation and syringobulbia. 3304 16


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