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B Mokri

Publications and source records attributed to B Mokri.

At least 37 records · Page 2Linked to original sources
PubMed1998

Acquired Chiari I malformation secondary to spontaneous spinal cerebrospinal fluid leakage and chronic intracranial hypotension syndrome in seven cases.

OBJECT: Spontaneous spinal cerebrospinal fluid (CSF) leakage with development of the intracranial hypotension syndrome and acquired Chiari I malformation due to lumbar spinal CSF diversion procedures have both been well described. However, concomitant presentation of both syndromes has rarely been reported. The object of this paper is to present data in seven cases in which both syndromes were present. Three illustrative cases are reported in detail. METHODS: The authors describe seven symptomatic cases of spontaneous spinal CSF leakage with chronic intracranial hypotension syndrome in which magnetic resonance (MR) images depicted dural enhancement, brain sagging, loss of CSF cisterns, and acquired Chiari I malformation. CONCLUSIONS: This subtype of intracranial hypotension syndrome probably results from chronic spinal drainage of CSF or high-flow CSF shunting and subsequent loss of brain buoyancy that results in brain settling and herniation of hindbrain structures through the foramen magnum. Of 35 cases of spontaneous spinal CSF leakage identified in the authors' practice over the last decade, MR imaging evidence of acquired Chiari I malformation has been shown in seven. Not to be confused with idiopathic Chiari I malformation, ideal therapy requires recognition of the syndrome and treatment directed to the site of the spinal CSF leak.

Adult↗
PubMed1997

Aortic dissection decades following internal carotid artery dissection--report of two cases.

Recurrent dissections involving carotid, vertebral, or renal arteries have been described in patients with spontaneous cervical artery dissections, with a maximal interval between dissections of fourteen years. The authors describe 2 patients in whom aortic dissections developed twenty-five and forty years, respectively, following carotid artery dissections. These 2 patients constituted 8% of the total number of patients from Rochester, Minnesota, who were diagnosed with aortic dissection between 1987 and 1992. The first patient, a forty-five-year-old woman, presented in 1948 with right neck pain and headache, associated with several episodes of transient numbness of the right face and numbness and clumsiness of the left upper and lower extremities. Examination showed right miosis. Angiography showed a stenosis of the extracranial right internal carotid artery beginning several centimeters from the bifurcation. She died at age eighty-five from an aortic dissection. The second patient, a thirty-eight-year-old man, noted left orbital and frontotemporal headaches and drooping of the left eyelid in 1962. Examination showed left oculosympathetic palsy. Angiography showed stenosis and an aneurysm in the midportion of the extracranial left internal carotid artery. He died at age sixty-three from an aortic dissection. These cases suggest that following a carotid artery dissection the risk of a recurrent arterial dissection may remain elevated for a prolonged period of time and the recurrent dissection may involve the aorta.

Adult↗
PubMed1997

Syndrome of orthostatic headaches and diffuse pachymeningeal gadolinium enhancement.

OBJECTIVE: To characterize clinical and imaging features, biopsy findings, etiologic factors, and outcome in the syndrome of intracranial hypotension, headaches, and diffuse pachymeningeal gadolinium enhancement on magnetic resonance imaging (MRI). MATERIAL AND METHODS: We describe our experience with 26 consecutive patients with orthostatic headaches and diffuse pachymeningeal gadolinium enhancement, for all of whom clinical, imaging, and follow-up data were available. For 10 patients who had undergone meningeal biopsy, slide material was also reviewed. RESULTS: The 15 men and 11 women ranged from 24 to 76 years of age. All 26 patients had postural headaches; in 22 patients, the headaches were completely alleviated by recumbency. Nausea or emesis, neck pain, horizontal diplopia, changes in hearing, photophobia, upper limb pains or paresthesias, visual blurring, or dysgeusia was noted in some of the patients. Cardinal MRI features were diffuse pachymeningeal gadolinium enhancement (100%), subdural collections of fluid (69%), and evidence of descent of the brain (62%) that sometimes resembled type I Chiari malformation. Cerebrospinal fluid (CSF) opening pressures were 40 mm or less in only 46%. In three patients, CSF pressures were consistently no less than 90 and as high as 130 mm of water. A variable pleocytosis of 5 or more cells/mm3 was noted in 15 patients (more than 40 cells/mm3 in 4 patients). A variable increase in CSF protein was noted in at least one spinal tap in 23 patients. Six patients had overdraining CSF shunts; CSF leak was documented in another 11 patients. Shunt revision or ligation and surgical correction of the leak led to a resolution of the clinical and MRI abnormalities in all cases thus treated. Improvement occurred with epidural blood patch in four patients. Three of the 12 patients treated supportively have remained symptomatic. Histologically, a thin subdural zone of fibroblasts and thin-walled vessels was noted in an amorphous matrix. Two patients with prolonged symptoms had a more pronounced proliferative reaction. CONCLUSION: The syndrome of low-pressure headaches and pachymeningeal gadolinium enhancement is being recognized with increasing frequency. The source of the CSF leak can be demonstrated in many patients. Meningeal abnormalities are likely attributable to decreased CSF volume and hydrostatic CSF pressure changes. The prognosis is typically good.

Adult↗
PubMed1996

Intracranial aneurysms and cervicocephalic arterial dissections associated with congenital heart disease.

OBJECTIVE: The number of patients with congenital heart disease who survive to adolescence and adulthood continues to increase. We review our experience with noninfectious intracranial aneurysms and cervicocephalic arterial dissections in patients with congenital heart disease, expanding the clinical spectrum of the cerebrovascular abnormalities that may be encountered in this group of patients. METHODS: All patients with congenital heart disease and intracranial aneurysms of cervicocephalic arterial dissections, who were evaluated at the Mayo Clinic between 1969 and 1992, were identified. RESULTS: Congenital heart disease was diagnosed in 3 (8%) of 36 children with intracranial aneurysms, in 5 (0.3%) of 1994 adults with intracranial aneurysms, in 1 (4%) of 25 children with cervicocephalic arterial dissections, and in 5 (2%) of 250 adults with cervicocephalic arterial dissections. The mean age of the 14 patients was 32 years (range, 13-58 yr). The congenital heart disorders consisted of complex cardiac anomalies in three patients (truncus arteriosus, transposition of the great arteries, and tricuspid atresia in one patient each), pulmonic valve or arterial stenosis in two patients, aortic coarctation in four patients, and bicuspid aortic valve in five patients. Only one patient had an intracranial aneurysm and coarctation of the aorta. CONCLUSION: Individuals with a variety of congenital heart disorders may be at an increased risk of intracranial aneurysm development and cervicocephalic arterial dissection, particularly in adolescence. The muscular arteries of the head and neck are derived from neural crest cells and the neural crest is also of major importance in early cardiac development, suggesting that an abnormality of the neural crest may be the common pathogenetic factor explaining this association.

Adolescent↗
PubMed1996

Butterfly vertebra.

A 41-year-old man with low back pain and lumbar disc disease was found to have L-3 butterfly vertebra on imaging studies as an incidental finding. This uncommon congenital anomaly of the vertebral column is usually asymptomatic and of no clinical significance. Awareness of this deformity and its imaging features is important diagnostically.

Abnormalities, Multiple↗
PubMed1996

Recurrent spontaneous arterial dissections: risk in familial versus nonfamilial disease.

BACKGROUND AND PURPOSE: Among patients with spontaneous cervical artery dissections, the risk of recurrent arterial dissection is relatively low at 1% per year, but this risk may be higher for patients with a family history of arterial dissections. We compared the risk of a recurrent arterial dissection in patients with familial versus non familial disease. METHODS: Long-term follow-up was established in 200 patients (104 women and 96 men with a mean age of 44.9 years) with spontaneous cervical artery dissections evaluated at a single institution between 1970 and 1990. RESULTS: Among the 200 patients, 10 (5%) were identified who had a family history of spontaneous arterial dissections. In a multivariate analysis, family history was the only significant variable associated with the risk of recurrent dissection (X2=15.51; P=.0001). A recurrent arterial dissection was identified in 5 (50%) of the 10 patients with familial disease compared with 11 (5.8%) of the 190 patients with nonfamilial disease, with an estimated relative risk of 6.3 (95% confidence interval, 2.2 to 18.3; P=.0007). CONCLUSIONS: Among patients with spontaneous cervical artery dissections, a family history of arterial dissection is an important risk factor for the development of a recurrent arterial dissection.

Adolescent↗
PubMed1996

Cranial nerve palsy in spontaneous dissection of the extracranial internal carotid artery.

Cranial nerve palsy was present in 23 of 190 consecutive adult patients (12%) with spontaneous dissection of the extracranial internal carotid artery. Ten patients (5.2%) had a syndrome of lower cranial nerve palsies (with invariable involvement of cranial nerve XII with or without additional involvement of cranial nerves XI, X, and IX), seven (3.7%) had palsy of cranial nerve V, and five (2.6%) had a syndrome of ocular motor palsies. Palsy of cranial nerve VIII and ischemic optic neuropathy occurred in one patient each. Three patients had dysgeusia without other cranial nerve involvement, presumably due to involvement of the chorda tympani nerve. Headache or face pain (often unilateral) was present in 83% of patients. Other associated manifestations were cerebral ischemic symptoms, bruits, or oculosympathetic palsy. In one patient, cranial nerve palsy was the only manifestation of internal carotid artery dissection, and in another patient, the disease presented only as a palsy of cranial nerve XII and oculosympathetic palsy. In six patients, a syndrome of hemicrania and ipsilateral cranial nerve palsy was the sole manifestation of internal carotid artery dissection. Cranial nerve palsy is not rare in internal carotid artery dissection. Compression or stretching of the nerve by the expanded artery may explain some but not all of the palsies. An alternative mechanism is likely interruption of the nutrient vessels supplying the nerve.

Adult↗
PubMed1996

Spontaneous spinal cerebrospinal fluid leaks and intracranial hypotension.

Spinal cerebrospinal fluid (CSF) leaks are often implicated as the cause of the syndrome of spontaneous intracranial hypotension, but they have rarely been demonstrated radiographically or surgically. The authors reviewed their experience with documented cases of spinal CSF leaks of spontaneous onset in 11 patients including their surgical observations in four of the patients. The mean age of the six women and five men included in the study was 38 years (range 22-51 years). All patients presented with a postural headache; however, most had additional symptoms, including nausea, emesis, sixth cranial-nerve paresis, or local back pain at the level of the CSF leak. All patients underwent indium-111 radionucleotide cisternography or computerized tomographic (CT) myelography. The location of the spontaneous CSF leak was in the cervical spine in two patients, the cervicothoracic junction in three patients, the thoracic spine in five patients, and the lumbar spine in one patient. The false negative rate for radionucleotide cisternography was high (30%). Subdural fluid collections, meningeal enhancement, and downward displacement of the cerebellum, resembling a Chiari I malformation, were commonly found on cranial imaging studies. In most patients, the symptoms resolved in response to supportive measures or an epidural blood patch. Leaking meningeal diverticula were found to be the cause of the CSF leak in four patients who underwent surgery. In three patients these diverticula could be ligated with good result but in one patient an extensive complex of meningeal diverticula was found to be inoperable. Two patients had an unusual body habitus and joint hypermobility, and two other patients had suffered a spontaneous retinal detachment at a young age. In conclusion, spontaneous spinal CSF leaks are uncommon, but they are increasingly recognized as a cause of spontaneous intracranial hypotension. Most spinal CSF leaks are located at the cervicothoracic junction or in the thoracic spine, and they may be associated with meningeal diverticula. The radiographic study of choice is CT myelography. The disease is usually self-limiting, but in selected cases our experience with surgical ligation of leaking meningeal diverticula has been satisfactory. An underlying connective tissue disorder may be present in some patients with a spontaneous spinal CSF leak.

Adult↗
PubMed1995

The trends in incidence of primary brain tumors in the population of Rochester, Minnesota.

A number of reports have suggested an increasing incidence of primary brain tumors, especially malignant astrocytomas, in the elderly population. To investigate this issue, we analyzed the incidence and temporal trends of primary intracranial neoplasms diagnosed in the population of Rochester, Minnesota, over the 40 years between 1950 and 1990. The incidence of symptomatic primary brain tumors (excluding patients diagnosed incidentally at autopsy and by neuroimaging studies) increased from 9.5 per 100,000 population per year in 1950 to 1969 to 12.5 per 100,000 per year in 1970 to 1989; this change was not statistically significant (chi 2 trend, 1.89; p = 0.17). While the incidence of pituitary adenomas increased significantly between the two periods (chi 2 trend, 4.44; p = 0.04), the incidence trends of all gliomas, malignant astrocytomas, and meningiomas showed no change among persons younger than 65 years as well as those 65 years and older. The number of patients incidentally found to have neoplasms by neuroimaging studies increased in the recent 20-year period (chi 2 trend, 4.08; p = 0.04). The average age- and sex-adjusted incidence rates per 100,000 per year during the study period in the population of Rochester, Minnesota, for symptomatic tumors were 5.0 for all gliomas, 3.3 for malignant astrocytomas, 2.0 for meningiomas, and 2.4 for pituitary adenomas. In conclusion, our data indicate that the reported increase in the incidence of primary brain tumors is an artifact of improvement in diagnostic technology and practice.

Adenoma↗
PubMed1995

Familial aorto-cervicocephalic arterial dissections and congenitally bicuspid aortic valve.

BACKGROUND: A primary arteriopathy is often implicated in the etiology of spontaneous cervicocephalic arterial dissections, but its exact nature usually remains unknown. We describe the familial occurrence of spontaneous arterial dissections and congenitally bicuspid aortic valve (BAV) and propose a common developmental defect in these families. SUMMARY OF REPORT: In the first family, a 63-year-old man suffered an extracranial internal carotid artery (ICA) dissection, and his 43-year-old cousin with BAV suffered an intracranial vertebral artery (VA) dissection. Two other family members had pathologically proven BAV. In the second family, a 31-year-old woman suffered bilateral extracranial ICA and VA dissections. Her father, at age 46, suffered an aortic dissection associated with cystic medial necrosis and BAV. Her paternal uncle died from an aortic dissection at age 59. In the third family, a 39-year-old woman suffered extracranial ICA and VA dissections, and her brother died at age 48 from an aortic dissection associated with a BAV. CONCLUSIONS: The familial occurrence of spontaneous arterial dissections and BAV suggests a common developmental defect. The aortic valvular cusps and the arterial media of the aortic arch and its branches are derived from neural crest cells, suggesting that a neural crest defect may be the underlying abnormality in these families.

Adult↗
PubMed1995

Meningeal biopsy in intracranial hypotension: meningeal enhancement on MRI.

Intracranial hypotension is a cause of diffuse enhancement of the pachymeninx with gadolinium, which often is associated with subdural fluid collections. We reviewed the results of meningeal biopsy in six patients with intracranial hypotension and diffuse pachymeningeal enhancement to correlate the MRI findings with histopathologic observations and to explain the abnormalities seen on MRI. Grossly, the dura mater was unremarkable in all patients, as were the leptomeninges, except for one patient with prolonged (18 months) intracranial hypotension in whom the arachnoid was thickened and opaque. Microscopically, the dura mater was entirely normal on its epidural aspect; however, a fairly thin zone of fibroblasts and thin-walled small blood vessels in an amorphous matrix was noted on the subdural aspect. In the patient with longstanding symptoms, diffuse benign arachnoidal cell proliferation was also noted, probably a reaction triggered by longstanding changes in the subdural area, as noted in the five other patients. There was no evidence of inflammation, infection, or metastatic neoplasia. These findings suggest that in intracranial hypotension, the dural-meningeal abnormalities probably represent reactive secondary phenomena, likely related to hydrostatic changes in the CSF, and not a primary meningeal process.

Aged↗
PubMed1995

Headache and neck pain in spontaneous internal carotid and vertebral artery dissections.

We studied the characteristics of headaches in 161 consecutive symptomatic patients with spontaneous dissections of the internal carotid artery (n = 135) or the vertebral artery (n = 26). For patients with internal carotid artery dissection (ICAD), the mean age was 47 years and for those with vertebral artery dissection (VAD), 40.7 years. A history of migraine was present in 18% of the ICAD group and in 23% of the VAD group. Headache was reported by 68% of the patients with ICAD and by 69% of those with VAD, and, when present, it was the initial manifestation in 47% of those with ICAD and in 33% of those with VAD. Ten percent of patients with ICAD had eye, facial, or ear pain without headache. The median interval from onset of headache to development of other neurologic manifestations was 4 days for the ICAD group and 14.5 hours for the VAD group. For all dissections, headaches typically were ipsilateral to the side of dissection. In the ICAD group, headaches were limited to the anterior head in 60% of patients and were steady in 73% and pulsating in 25%. In the VAD group, headaches were distributed posteriorly in 83% of patients and were steady in 56% and pulsating in 44%. Neck pain was present in 26% of patients with ICAD (anterolateral) and in 46% of those with VAD (posterior). The median duration of the headache in patients with VAD and ICAD was 72 hours, but headaches became prolonged, persisting for months to years, in four patients with ICAD.

Adult↗
PubMed1994

Alpha 1-antitrypsin deficiency in intracranial aneurysms and cervical artery dissection.

The pathogenesis of ruptured intracranial aneurysms and cervical artery dissections is poorly understood but may be similar in these two disorders. We report four patients with alpha 1-antitrypsin deficiency who developed a ruptured intracranial aneurysm or spontaneous dissection of the cervical internal carotid artery. Three patients were heterozygous for the deficient allele (PiMZ or PiGZ) and one was homozygous (PiZZ). A deficiency of alpha 1-antitrypsin or one of the other protease inhibitors could result in degradation of the arterial wall through an imbalance between proteolytic enzymes and their inhibitors, thereby predisposing the arterial wall to dissection or aneurysm formation.

Adult↗
PubMed1994

Recurrent spontaneous cervical-artery dissection.

BACKGROUND: Spontaneous dissection of the internal carotid and vertebral arteries is increasingly recognized as a cause of ischemic stroke in young people. An underlying arteriopathy is often suspected in the pathogenesis of such dissection, but the frequency of recurrent dissection is unknown. METHODS: We describe the long-term follow-up of 200 consecutive patients (104 women and 96 men) with spontaneous cervical-artery dissections evaluated at the Mayo Clinic between 1970 and 1990. All diagnoses were confirmed by angiography. RESULTS: The mean age of the patients was 44.9 years (range, 16 to 76). Internal carotid arteries were affected in 150 patients, vertebral arteries in 37, and both in 13. Multivessel dissections were present in 28 percent of the patients. The mean follow-up was 7.4 years. Recurrent dissection occurred only in arteries not previously involved by dissection. A recurrent arterial dissection developed in 16 patients (8 percent)--within a month after the initial dissection in 4 patients (2 percent) and between 1.4 and 8.6 years later in 12 patients (a rate of 1 percent per year). The cumulative rate of recurrent dissection among patients followed for 10 years was 11.9 percent. Younger patients had a greater risk of recurrent dissection. CONCLUSIONS: Although dissections in multiple cervical vessels are common at presentation, after the first month the risk of recurrent dissection is only about 1 percent per year.

Adolescent↗