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Bahram Mokri

Publications and source records attributed to Bahram Mokri.

18 recordsLinked to original sources
PubMed2006

Incidence and outcome of cervical artery dissection: a population-based study.

BACKGROUND: Incidence rates for internal carotid artery dissection (ICAD) have been reported to be 2.6 to 2.9 per 100,000, but reliable epidemiologic data for vertebral artery dissection (VAD) are not available. OBJECTIVE: To determine the incidence rate of cervical artery dissection (CAD) in a defined population. METHODS: With IRB approval, we used the medical record linkage system of the Rochester Epidemiology Project to identify all patients diagnosed with spontaneous ICAD and VAD for the period of 1987-2003 in Olmsted County, MN. RESULTS: Of 48 patients with CAD, there were 32 patients with ICAD and 18 patients with VAD. In Olmsted County, the average annual incidence rate for ICAD was 1.72 per 100,000 population (95% CI, 1.13 to 2.32) and for VAD 0.97 per 100,000 population (95% CI, 0.52 to 1.4). The average annual incidence rate for CAD was 2.6 per 100,000 population (95% CI, 1.86 to 3.33). The most frequently encountered symptoms in CAD were head or neck pain (80%), cerebral ischemia (TIA or infarct) (56%), and Horner syndrome (25%). Good outcome (defined as modified Rankin score of 0 to 2) was seen in 92% of patients. No recurrence of dissection was observed during a mean 7.8 years of follow-up. CONCLUSIONS: Internal carotid artery dissection was detected approximately twice as frequently as vertebral artery dissection in the overall study, but in the latter half of the study period, vertebral artery and internal carotid artery dissection incidence rates were equivalent. The majority of cervical artery dissection patients in the community have excellent outcome, and contrary to many tertiary referral series, re-dissection is rare.

Adult↗
PubMed2006

Chorea as a manifestation of spontaneous CSF leak.

A 59-year-old man presented with orthostatic headaches, memory complaints, pronounced choreiform movements, and related hyperkinetic dysarthria and titubations. Head MRI findings were suggestive of CSF leak. CSF pressure was low. CT myelography documented CSF leak at the cervicothoracic junction. Targeted epidural blood patch led to resolution of symptoms, including complete disappearance of choreiform movements.

Blood Patch, Epidural↗
PubMed2006

Steroid-responsive encephalopathy associated with autoimmune thyroiditis.

BACKGROUND: Steroid-responsive encephalopathy associated with autoimmune thyroiditis (SREAT), often termed Hashimoto encephalopathy, is a poorly understood and often misdiagnosed entity. OBJECTIVE: To characterize the clinical, laboratory, and radiologic findings in patients with SREAT to potentially improve recognition of this treatable entity. DESIGN: Retrospective analysis of clinical features and diagnostic test data. SETTING: Two affiliated tertiary care referral institutions. PATIENTS: Twenty consecutive (6 male) patients diagnosed as having SREAT from 1995 to 2003. MAIN OUTCOME MEASURES: Clinical features and ancillary test findings associated with SREAT. RESULTS: The median age at disease onset was 56 years (range, 27-84 years). The most frequent clinical features were tremor in 16 (80%), transient aphasia in 16 (80%), myoclonus in 13 (65%), gait ataxia in 13 (65%), seizures in 12 (60%), and sleep abnormalities in 11 (55%). All patients were assigned an alternative misdiagnosis at presentation, most commonly viral encephalitis (n = 5), Creutzfeldt-Jakob disease (n = 3), or a degenerative dementia (n = 4). The most frequent laboratory abnormalities were increased liver enzyme levels in 11, increased serum sensitive thyroid-stimulating hormone levels in 11, and increased erythrocyte sedimentation rate in 5. In only 5 patients (25%) did cerebrospinal fluid abnormalities suggest an inflammatory process. Magnetic resonance imaging abnormalities believed to be related to the encephalopathy were present in 5 patients (26%). CONCLUSIONS: The clinical, laboratory, and radiologic findings associated with SREAT are more varied than previously reported. Misdiagnosis at presentation is common. This treatable syndrome should be considered even if the serum sensitive thyroid-stimulating hormone level and erythrocyte sedimentation rate are normal, the cerebrospinal fluid profile does not suggest an inflammatory process, and neuroimaging results are normal. Until the pathophysiologic mechanism of this and other autoimmune encephalopathies is better characterized, we believe that descriptive terms that reflect an association rather than causation are most appropriate for this syndrome.

Adult↗
PubMed2006

Surgical anatomy of dural defects in spontaneous spinal cerebrospinal fluid leaks.

OBJECTIVE: Spontaneous intracranial hypotension is typically caused by a spontaneous spinal cerebrospinal fluid (CSF) leak. The configuration of the related dural defects can be complex. We describe our experience with the surgical anatomy of these defects. METHODS: Thirteen consecutive patients with spontaneous spinal CSF leaks who underwent surgical exploration at Mayo Clinic between 1994 and 2003 were studied. All patients' records, imaging studies, detailed intraoperative findings, and outcomes were reviewed. RESULTS: There were four men and nine women with an average age of 40 years (range, 12-62 yr). Preoperative imaging studies revealed a single site of CSF leak in eight patients, two sites in three patients, and multiple sites in two patients. Intraoperatively, the exact site of leakage could not be found in four patients. Among the other nine patients, primary closure of a meningeal diverticulum was achieved in one patient. Significant regional attenuation of the dura prevented primary repair of the leak site in eight patients. Muscle, fibrin glue, and Gelfoam (Upjohn Co., Kalamazoo, MI) soaked in patient's own blood were commonly used to pack the epidural space in an attempt to seal the site of the leak. Ligation of two nonappendicular nerve roots allowed closure of the leak in one of these patients. Postoperatively, resolution of symptoms occurred in eight patients, significant improvement was noted in three patients, and only transient resolution in two. The mean duration of follow-up was 20.5 months. CONCLUSION: Surgery for closure of spontaneous spinal CSF leaks may not be straightforward. Even when extradural CSF leakage is discovered preoperatively by imaging studies, it may not always be possible to identify the exact site of the leakage intraoperatively. Furthermore, the anatomy of the dural defects may be complex and not amenable to primary closure. In such cases, the use of adjuvant techniques during surgical exploration may be effective.

Adolescent↗
PubMed2005

Amphiphysin autoimmunity: paraneoplastic accompaniments.

Amphiphysin-IgG was identified in 71 patients among 120,000 evaluated serologically for paraneoplastic autoantibodies. Clinical information was available for 63 patients. Cancer was detected in 50 (mostly limited), proven histologically in 46, and was imaged intrathoracically in 4 patients (lung, small-cell [27] and non-small cell [1]), breast [16] and melanoma [2]). Neurological accompaniments included (decreasing frequency): neuropathy, encephalopathy, myelopathy, stiff-man phenomena, and cerebellar syndrome. In a case examined neuropathologically, parenchymal T-lymphocyte infiltration (predominantly CD8(+)) was prominent in lower brainstem, spinal cord, and dorsal root ganglion. Coexisting paraneoplastic autoantibodies, identified in 74% of patients, predicted a common neoplasm and indicated other neuronal autoantigen targets that plausibly explained several neurological manifestations; for example, P/Q-type Ca(2+)-channel antibody with Lambert-Eaton syndrome (n = 5), anti-neuronal nuclear antibody type 1 with sensory neuronopathy (n = 7), K(+)-channel antibody with limbic encephalitis (n = 1) or neuromyotonia (n = 1), and collapsin response-mediator protein-5-IgG with optic neuritis (n = 3). Patients with isolated amphiphysin-IgG (n = 19) were more likely to be women (with breast cancer, p < 0.05) and to have myelopathy or stiff-man phenomena (p < 0.01). Overall, a minority of women (39%) and men (12%) had stiff-man phenomena. Only 10% of women (some with lung carcinoma) and 4% of men fulfilled diagnostic criteria for stiff-man syndrome.

Aged↗
PubMed2004

Syndrome resembling PSP after surgical repair of ascending aorta dissection or aneurysm.

Seven patients, after seemingly uncomplicated surgery for ascending aorta aneurysm or dissection, with or without aortic valve replacement, developed an unusual and fairly stereotyped biphasic neurologic disorder without imaging evidence of related cerebral ischemia or infarct. The initial phase was mild, nonprogressive, or receding. The latent and progressive phase closely resembled a progressive supranuclear palsy phenotype. The disorder may prove to be self-limiting but leaves the patients with considerable neurologic deficits.

Adult↗
PubMed2004

Spontaneous low cerebrospinal pressure/volume headaches.

The majority, if not all, of the cases of spontaneous intracranial hypotension result from spontaneous cerebrospinal fluid (CSF) leaks. The disorder has a broad clinical and imaging spectrum with substantial variability in clinical and imaging features, in CSF findings, and in response to treatment. Headache is the most common symptom and is typically orthostatic, but with chronicity the orthostatic features may blur into a chronic, lingering headache. Other clinical features include neck pain, nausea, emesis, interscapular pain, diplopia, dizziness, change in hearing, visual blurring, radicular upper extremity symptoms, and a variety of other, but much less common, manifestations. The most common imaging feature is diffuse pachymeningeal gadolinium enhancement. Other manifestations include imaging evidence of sinking of the brain, subdural fluid collections, enlargement of the pituitary, engorgement of venous sinuses, and engorgement of epidural venous plexus. CSF opening pressure is typically low and CSF analysis may be normal or show increased protein concentration and a primarily lymphocytic pleocytosis. No longer can the entity be simply equated with post-spinal puncture headaches. The pathogenetic core and the independent variable is decrease in CSF volume, whereas clinical imaging and CSF findings, including CSF opening pressures, are all variables dependent on the loss of CSF volume. Many patients respond well to treatment, but some present stubborn therapeutic challenges. A subgroup of patients with orthostatic headaches is gradually recognized who have disorders other than CSF leaks.

Blood Patch, Epidural↗
PubMed2004

Low cerebrospinal fluid pressure syndromes.

Only 12 years ago the first report on pachymeningeal gadolinium enhancement in low-pressure headaches appeared in the literature. In this short interval the enormous impact of MRI on so-called "spontaneous intracranial hypotension" and "low-pressure head-aches" has become obvious. A much broader clinical and imaging spectrum of the disease is now recognized and a substantially larger number of patients is diagnosed. In the past decade there has been remarkable progress in understanding this disorder and its associated cerebrospinal fluid (CSF) dynamics. Some of the older concepts or presumptions have been challenged while novel observations continue to appear in the literature. We are still in the learning phase.

Brain↗
PubMed2003

Orthostatic headaches without CSF leak in postural tachycardia syndrome.

Four women age 17 to 28 years presented with orthostatic headaches as the most prominent feature of their symptom complex. None had CSF leak or intracranial hypotension. Autonomic studies showed evidence of orthostatic intolerance with tachycardia in all cases. Treatment of orthostatic intolerance, mainly with volume expansion, was only partially effective. Orthostatic headaches are not always caused by CSF leak or supine intracranial hypotension. Occasionally they may be the major clinical manifestation of postural tachycardia syndrome or orthostatic intolerance.

Adolescent↗
PubMed2003

Headaches caused by decreased intracranial pressure: diagnosis and management.

PURPOSE OF REVIEW: More patients with spontaneous intracranial hypotension are now being diagnosed, and it is realized that most cases result from spontaneous cerebrospinal fluid leaks. A broader clinical and imaging spectrum of the disorder is recognized. This paper reviews new insights into the variability of clinical manifestations, imaging features, etiological factors, anatomy of leaks, and implications of these in patient management. RECENT FINDINGS: Spontaneous intracranial hypotension should not be equated with post-lumbar puncture headaches. In a substantial minority of patients, headaches are not orthostatic and may mimic other types of headache. Additional diverse neurological manifestations may dominate the clinical picture and patients may occasionally have no headache at all. Reports on unusual presentations of the disorder continue to appear in the literature. Furthermore, additional imaging features of cerebrospinal fluid leaks are recognized. High-flow and slow-flow leaks may present diagnostic challenges, and require modification of diagnostic studies aimed at locating the site of the leak. Stigmata of connective tissue abnormality, especially abnormalities of fibrillin and elastin, are seen in a notable minority of patients, pointing to weakness of the dural sac as one of the etiological factors. After treatment of spontaneous intracranial hypotension, surgically or by epidural blood patch, a rebound and self-limiting intracranial hypertension may sometimes develop. SUMMARY: In the past decade, interest in spontaneous intracranial hypotension has been rekindled, with a substantial growth of knowledge on various aspects of the disorder. We are in the learning phase, and new information will probably appear in the future, with notable diagnostic and therapeutic implications.

Cerebrospinal Fluid↗
PubMed2003

Dynamic CT myelography: a technique for localizing high-flow spinal cerebrospinal fluid leaks.

In some patients with spontaneous spinal CSF leaks, leaks are numerous or tears are so large that extrathecal myelographic contrast material is seen at multiple levels during CT, making identification of their source impossible. This study introduces a dynamic CT myelographic technique that provides high temporal and spatial resolution. In this technical note, we describe the utility of this technique in four patients with challenging high-flow spinal CSF leaks.

Cerebrospinal Fluid↗
PubMed2002

Spontaneous CSF leaks: underlying disorder of connective tissue.

Of 58 consecutive patients with spontaneous CSF leaks, nine exhibited features of connective tissue disorder. One had Marfan's syndrome. Five additional patients had hyperflexible joints, of whom four had arachnodactyly, four were tall and slender, two had hyperextensible skin, and one had a strong family history of abdominal aorta aneurysms. Retinal detachment at a young age was noted in two. One patient had bilateral carotid dissections. A dural weakness may predispose patients to spontaneous CSF leak.

Adult↗
PubMed2002

Headaches in cervical artery dissections.

Internal carotid artery (ICA) and vertebral artery (VA) dissections are among the common causes of stroke in middle-aged and young adults. The spectrum of clinical presentations of these dissections is broad. Many patients, especially those with ICA dissections, may never develop a stroke.

Carotid Arteries↗
PubMed2002

Rarity of encephalopathy associated with autoimmune thyroiditis: a case series from Mayo Clinic from 1950 to 1996.

Corticosteroid-responsive encephalopathy associated with autoimmune thyroiditis (also called Hashimoto's encephalopathy) is a rare, life-threatening, treatable, and possibly autoimmune condition. We identified nine patients (with the diagnosis made after 1979) who had relapsing encephalopathy compatible with previous reports of Hashimoto's encephalopathy and no other identifiable cause of encephalopathy at Mayo Clinic Rochester. Of these nine patients, three were clinically hypothyroid, four were subclinically hypothyroid, and two were euthyroid. Thyroid antibodies were positive in eight of eight patients in whom these measurements were made. Electroencephalographic abnormalities were identified in eight of the nine patients (89%). Magnetic resonance imaging (MRI) abnormalities considered etiologically related to encephalopathy were present in three patients (33%). An increased protein concentration was noted on cerebrospinal fluid examination in seven patients (78%). Of the six patients who received high-dose glucocorticoid therapy, 5 (83%) had improvement of neurologic symptoms. In conclusion, encephalopathy associated with autoimmune thyroiditis is rare but important to recognize because it may be responsive to high-dose glucocorticoid therapy. We believe that this condition is not caused by thyroid dysfunction or antithyroid antibodies but represents an association of an uncommon autoimmune encephalopathy with a common autoimmune thyroid disease. The term Hashimoto's encephalopathy is a misnomer and should not be used.

Adolescent↗
PubMed2002

Intracranial hypertension after treatment of spontaneous cerebrospinal fluid leaks.

Four patients, aged 10 to 44 years, with spontaneous cerebrospinal fluid (CSF) leaks and intracranial hypotension developed intracranial hypertension after treatment of their CSF leaks. The leak was at the spinal level in all patients (thoracic level, 2; lumbar level, 1; and undetermined, 1). One patient responded to an epidural blood patch. Three patients responded to surgery, of whom 2 had not responded to prior epidural blood patches. Treatment resulted in complete resolution of symptoms, including orthostatic headaches and disappearance of magnetic resonance imaging abnormalities. However, all patients later developed steady headaches different from their previous headaches. None had recurrence of magnetic resonance imaging abnormalities or any evidence of occlusion of cerebral venous sinuses. All had increased CSF opening pressures. One had bilateral papilledema, and another had no venous pulsations on examination of fundi. Follow-up was possible in 2 patients. One responded well to treatment with acetazolamide, and the other improved gradually and was asymptomatic within several months.

Adolescent↗
PubMed2002

Cervical artery dissections: evidence for redissection in previously dissected arteries: report of three cases.

OBJECTIVE AND IMPORTANCE: Among patients with spontaneous internal carotid artery (ICA) or vertebral artery dissections, there is a small but significant risk of development of dissection in an uninvolved ICA or vertebral artery at some time in the future. Recurrence of dissection in a previously dissected and healed ICA or vertebral artery is very rare. We retrospectively reviewed more than 400 cases of cervical artery dissections in our cerebrovascular records for 1970 through 2000. CLINICAL PRESENTATION: Three patients with recurrence of dissection in previously dissected and presumably healed ICAs were identified. All three patients were women. Their ages at the time of the initial dissection were 42, 38, and 31 years. Initial ICA dissections were bilateral in all cases, occurring simultaneously in one case and 1 year apart in the other two cases. All dissections were spontaneous. Redissection of a previously dissected and healed ICA in these three patents occurred 11, 6, and 12 years later, respectively. Two patients had fibromuscular dysplasia. CONCLUSION: Our experience with these three cases, from among more than 400 cases of cervical artery dissections treated at our institution, and the very limited number of reported cases in the literature indicate the rarity of recurrence of dissection in previously dissected and healed cervical arteries. An underlying arteriopathy may be suspected in many of these cases. Management requires medical and sometimes surgical approaches.

Adult↗