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Ten patients with clinically probable brainstem stroke presenting primarily as acute dysphagia but without visible brainstem abnormality by MRI are described. The patients were evaluated with neurologic examinations, cinepharyngoesophagography, and brain MRI studies. Each patient solely or predominately experienced sudden pharyngeal dysphagia, and additional symptoms or signs other than dysphonia or dysarthria were scarce. Small vessel disease or cardiac embolism were the apparent causes of what appear to have been very discrete brainstem strokes in these patients. Acute pharyngeal dysphagia can be the sole or primary manifestation of brainstem stroke. A negative MRI study should not preclude consideration of this diagnosis, if brainstem stroke is otherwise clinically probable. [ABSTRACT FROM AUTHOR]

Background: It is widely believed that pregnancy increases the risk of stroke, but there are few data available to quantify that risk.Methods: We identified all female patients 15 through 44 years of age in central Maryland and Washington, D.C., who were discharged from any of 46 hospitals in the study area in 1988 or 1991. Two neurologists reviewed each case, using data from the women's medical records. We determined whether the women had been pregnant at the time of the stroke or up to six weeks before it occurred. For purposes of this analysis, the six-week period after pregnancy could begin with an induced or spontaneous abortion or with the delivery of a live or stillborn child.Results: Seventeen cerebral infarctions and 14 intracerebral hemorrhages occurred in women who were or had recently been pregnant (pregnancy-related strokes), and there were 175 cerebral infarctions and 48 intracerebral hemorrhages that were not related to pregnancy. For cerebral infarction, the relative risk during pregnancy, adjusted age and race, was 0.7 (95 percent confidence interval, 0.3 to 1.6), but it increased to 8.7 for the postpartum period (after a live birth or stillbirth) (95 percent confidence interval, 4.6 to 16.7). For intracerebral hemorrhage, the adjusted relative risk was 2.5 during pregnancy (95 percent confidence interval, 1.0 to 6.4) but 28.3 for the postpartum period (95 percent confidence interval, 13.0 to 61.4). Overall, for either type of stroke during or within six weeks after pregnancy, the adjusted relative risk was 2.4 (95 percent confidence interval, 1.6 to 3.6), and the attributable, or excess, risk was 8.1 strokes per 100,000 pregnancies (95 percent confidence interval, 6.4 to 9.7).Conclusions: The risks of both cerebral infarction and intracerebral hemorrhage are increased in the six weeks after delivery but not during pregnancy itself. [ABSTRACT FROM AUTHOR]

Background and Purpose: lipoprotein (a) (lp (a)) is a lipid-containing particle similar to LDL which has been found in atherosclerotic plaque. The role of lp (a) in ischemic stroke remains controversial, but some studies suggest lp (a) is particularly important as a risk factor for stroke in young adults. We investigated the role of lp (a) as a risk factor for stroke in young women enrolled in the Stroke Prevention in Young Women Study., Methods: subjects were participants in a population-based, case-control study of risk factors for ischemic stroke in young women. Cases were derived from surveillance of 59 regional hospitals in the central Maryland, Washington DC, Pennsylvania and Delaware area. Lp (a) was measured in 110 cases and 216 age-matched controls. Demographics, risk factors, and stroke subtype were determined by interview and review of medical records., Results: lp (a) values were higher in blacks than whites, but within racial groups, the distribution of lp (a) values was similar between cases and controls. After adjustment for age, race, hypertension, diabetes, cigarette smoking, coronary artery disease, total cholesterol and HDL cholesterol, the odds ratio for an association of lp (a) and stroke was 1.36 (95% CI 0.80-2.29). There was no dose-response relationship between lp (a) quintile and stroke risk. Among stroke subtypes, only lacunar stroke patients had significantly elevated lp (a) values compared to controls., Conclusions: we found no association of lp (a) with stroke in a population of young women with ischemic stroke. Small numbers of patients limit conclusions regarding risk in ischemic stroke subtypes, but we could not confirm previous suggestions of an association of lp (a) with atherosclerotic stroke in young adults.

Purpose: To determine the distribution and correlates of elevated total homocyst(e)ine (tHcy) concentration in a population of premenopausal black and white women., Methods: Data from the Stroke Prevention in Young Women Study (N = 304), a population-based study of risk factors for stroke in women aged 15-44 years of age, were used to determine the distribution and correlates of elevated tHcy in black (N = 103) and white women (N = 201)., Results: The mean tHcy level for the population was 6.58 micromol/L (range 2.89-26.5 micromol/L). Mean tHcy levels increased with age, cholesterol level, alcohol intake, and number of cigarettes smoked (all: p < 0.05). There were no race differences (mean tHcy 6.72 micromol/L among blacks and 6.51 micromol/L among whites; p = 0.4346). Regular use of multivitamins and increasing education was associated with significant reductions in tHcy concentration. Approximately 13% of the sample had elevated tHcy levels, defined as a tHcy concentration > or = 10.0 micromol/L. Multivariate-adjusted correlates of elevated tHcy included education > 12 vs. < or = 12 (odds ratio [OR] = 0.4, 95% confidence interval [CI] = 0.2-0.8); smoking > or = 20 cigarettes/day vs. nonsmokers (OR = 2.8, 95% CI = 1.1-7.3); and the regular use of multivitamins (OR = 0.4, 95% CI = 0.2-0.9)., Conclusions: These results suggest that a substantial proportion of healthy young premenopausal women have tHcy levels that increase their risk for vascular disease. A number of potentially modifiable behavioral and environmental factors appear to be significantly related to elevated tHcy levels in young women.

A polymorphism associated with a thermolabile variant (C677T) of the enzyme methylenetetrahydrofolate reductase has been associated with both elevated total homocysteine (tHcy) levels and risk for cardiovascular disease. Data from the Stroke Prevention in Young Women Study were used to determine the prevalence of the C677T genotype and to assess whether environmental factors modified the association between genotype and tHcy concentration. The C677T genotype prevalence was 80% -/-, 20% +/-, and 0% +/+ among 46 African-American women; and 39% -/-, 53% +/-, and 8% +/+ among 77 white women (P < 0.01). There was a trend toward higher tHcy levels in African-American women with the +/- genotype when compared with the -/- genotype (6.9 mumol/L vs 5.3 mumol/L respectively, p = 0.10); no association was found among the white women (6.0 mumol/L, -/-; 4.5 mumol/L, +/-; and 6.2 mumol/L, +/+; p = 0.67). Among African American women, those who smoked and were +/- genotype had the highest tHcy levels (8.0 mumol/L); while among white women, those who smoked and were -/- had the highest tHcy levels (8.1 mumol/L). Despite being hampered by a limited sample size, the thermolabile allele is significantly less common among African-American than white women. The association between genotype and tHcy concentration is influenced by smoking and multivitamin use.

Maintaining nutrition in patients with neurologic disorders can be a difficult task. The best results can be expected with individual treatment plans based on a thorough knowledge of the disease process and carried out by a multidisciplinary team working in concert.

Migraine is conventionally regarded as a specific type of headache with a small set of associated neurologic symptoms. Yet, the true scope and frequency of migrainous phenomena are much greater than is generally acknowledged. Six cases are presented to illustrate some of the diverse manifestations of migraine: (1) transient global amnesia, (2) persistent visual phenomena, (3) migraine and seizures, (4) vestibular dysfunction, (5) hearing loss, and (6) migraine masquerading as multiple sclerosis. These and other migraine-associated neurologic symptoms are discussed in relation to previous reports in the literature. Pitfalls in recognizing the diverse manifestations of migraine, which are often underdiagnosed or misdiagnosed, are reviewed. Whether or not the established diagnostic criteria for migraine are too strict, other factors contributing to failure to identify migraine when it causes nonclassic neurologic symptoms include: (1) the belief that these are rare, (2) lack of an objective measure for migraine, (3) inadequate attention to the positive diagnostic features of migraine in the absence of headache, (4) confusion regarding prior migraine history, and (5) dismissing the diagnosis because of reported failure of prior migraine treatment. Recognition of the diversity of migraine helps avoid unnecessary testing and opens the door to effective treatment.

The role of cricopharyngeal (CP) myotomy in the management of neurogenic oropharyngeal dysphagia remains controversial. A review of the literature regarding outcomes of CP myotomy for dysphagia in the setting of a variety of neurological disorders indicates a preponderance of favorable results. There are several potential explanations for reported improvement after CP myotomy for neurogenic dysphagia, including the possibility that it is an effective treatment, at least for selected patients. If this is true, appropriate selection criteria for this treatment of neurogenic dysphagia may include (1) intact voluntary initiation of swallowing, (2) adequate propulsive force generated by the tongue and pharyngeal constrictors, (3) videofluorographic demonstration of obstruction to bolus flow at the CP segment (rather than merely retention in the pharyngeal recesses), (4) manometric evidence of relatively elevated CP pressure in relation to the pharynx, and (5) relatively favorable neurological prognosis. The effectiveness and safety of CP myotomy for patients with neurogenic dysphagia are unlikely to be resolved without a prospective, controlled multicenter study enrolling patients who meet such criteria.

Oropharyngeal dysphagia due to iatrogenic neurological dysfunction may relate to either medication side effects or surgical complications. There are several general mechanisms by which neurological side effects of medications can cause or aggravate oropharyngeal dysphagia. These include decreased level of arousal, direct suppression of brainstem swallowing regulation, movement disorders (dyskinesias, dystonias, and parkinsonism), neuromuscular junction blockade, myopathy, oropharyngeal sensory impairment, and disturbance of salivation. Postsurgical oropharyngeal dysphagia due to neurological dysfunction has been described in association with carotid endarterectomy, esophageal cancer surgery, anterior cervical fusion, and ventral rhizotomy for spasmodic torticollis. A potential explanation for oropharyngeal dysphagia following these surgical procedures is intraoperative mechanical disruption of the innervation of the pharyngeal constrictor muscles by the pharyngeal plexus. Posterior fossa and skull base surgery can lead to dysphagia as a result of intraoperative damage to brainstem centers and/or cranial nerves involved in swallowing. Perioperative stroke is the most likely explanation for oropharyngeal dysphagia appearing acutely following surgery, especially if the type of surgery predisposes to embolism or hypoperfusion.

The potential causes of neurogenic oropharyngeal dysphagia in cases in which the underlying neurologic disorder is not readily apparent are discussed. The most common basis for unexplained neurogenic dysphagia may be cerebrovascular disease in the form of either confluent periventricular infarcts or small, discrete brainstem stroke, which may be invisible by magnetic resonance imaging. The diagnosis of occult stroke causing pharyngeal dysphagia should not be overlooked, because this diagnosis carries important treatment implications. Motor neuron disease producing bulbar palsy, pseudobulbar palsy, or a combination of the two can present as gradually progressive dysphagia and dysarthria with little if any limb involvement. Myopathies, especially polymyositis, and myasthenia gravis are potentially treatable disorders that must be considered. A variety of medications may cause or exacerbate neurogenic dysphagia. Psychiatric disorders can masquerade as swallowing apraxia. The basis for unexplained neurogenic dysphagia can best be elucidated by methodical evaluation including careful history, neurologic examination, videofluoroscopy of swallowing, blood studies (CBC, chemistry panel, creatine kinase, B12, thyroid screening, and anti-acetylcholine receptor antibodies), electromyography, and magnetic resonance imaging (MRI) of the brain, plus additional procedures such as lumbar puncture and muscle biopsy as indicated. Little is known about aging and neurogenic dysphagia, specifically the relative contributions of natural age-related changes in the oropharynx and of diseases of the elderly, including periventricular MRI abnormalities, in producing dysphagia symptoms and videofluoroscopic abnormalities in this population.

Neurogenic dysphagia results from sensorimotor impairment of the oral and pharyngeal phases of swallowing due to a neurologic disorder. The symptoms of neurogenic dysphagia include drooling, difficulty initiating swallowing, nasal regurgitation, difficulty managing secretions, choke/cough episodes while feeding, and food sticking in the throat. If unrecognized and untreated, neurogenic dysphagia can lead to dehydration, malnutrition, and respiratory complications. The symptoms of neurogenic dysphagia may be relatively inapparent on account of both compensation for swallowing impairment and diminution of the laryngeal cough reflex due to a variety of factors. Patients with symptoms of oropharyngeal dysphagia should undergo videofluoroscopy of swallowing, which in the case of neurogenic dysphagia typically reveals impairment of oropharyngeal motor performance and/or laryngeal protection. The many causes of neurogenic dysphagia include stroke, head trauma, Parkinson's disease, motor neuron disease and myopathy. Evaluation of the cause of unexplained neurogenic dysphagia should include consultation by a neurologist, magnetic resonance imaging of the brain, blood tests (routine studies plus muscle enzymes, thyroid screening, vitamin B12 and anti-acetylcholine receptor antibodies), electromyography/nerve conduction studies, and, in certain cases, muscle biopsy or cerebrospinal fluid examination. Treatment of neurogenic dysphagia involves treatment of the underlying neurologic disorder (if possible), swallowing therapy (if oral feeding is reasonably safe to attempt) and gastrostomy (if oral feeding is unsafe or inadequate).

We studied the relative benefits of carbidopa/levodopa (25/100 mg and 50/200 mg) and propoxyphene (100 mg and 200 mg) in six subjects with periodic limb movements (PLM) using a randomized, double-blind, placebo-controlled, cross-over design with a 4-day placebo wash-out period between test medications. Each subject received, for successive 2-week periods, 4 days of placebo followed by 5 days of low-dose and then 5 days of high-dose medication. Outcome was measured with polysomnograms and sleep latency tests on the last night of the high dose, and leg activity was monitored for each night of the study. Carbidopa/levodopa normalized PLM and improved sleep, particularly in the first 3 hours in most subjects. Propoxyphene had marginal benefit and significantly decreased arousals and mildly decreased leg movements for leg activity monitoring, but not for PLM on the polysomnograms. Subjects reported better sleep and alertness on carbidopa/levodopa than on propoxyphene or placebo. Our results support the premise of a central dopaminergic abnormality in PLM.

Twenty patients with a remote history of poliomyelitis and recent or progressive dysphagia were evaluated with cinefluorography. Radiographic abnormalities were present in the pharynx in varying degrees in all but one of the patients. Findings included atrophy of the prevertebral soft tissues, unilateral or bilateral weakness of the tongue or soft palate, paresis or paralysis of the pharyngeal constrictor muscle, incomplete or absent epiglottic tilt, poor laryngeal elevation, poor laryngeal closure with laryngeal penetration, aspiration (often without a cough), and luminal narrowing at the cricopharyngeal level. Other structural lesions included a Zenker diverticulum in one patient, bilateral pharyngeal pouches in five, and a unilateral pouch in one. Additional structural lesions contributing to dysphagia were found in two other patients, including a focal stricture in the cervical esophagus in one patient and two stenotic rings in the distal esophagus in another. In four patients (one of whom had the Zenker diverticulum), the inferior constrictor muscle contracted forcibly above a prominent cricopharyngeus muscle, perhaps contributing to the formation of the diverticulum. It is important to examine postpolio patients with dysphagia carefully with dynamic imaging to assess the severity of decompensation and to detect other lesions that may be treatable. The information derived can be used to guide management.

Under normal circumstances, the act of swallowing adjusts to varying demands of different bolus characteristics and different head and neck postures. When the swallowing mechanism is impaired by disease, adjustment is essential to compensate for the impairment and allow swallowing. Evidence of adjustment can be demonstrated by cineradiography which provides important clues to the presence of underlying disease. When adjustment to disease is inadequate, swallowing decompensates. In this case, gross changes in swallowing performance are evident from clinical evaluation and cineradiography.

This paper describes the purpose and organization of a dedicated center at The Johns Hopkins Medical Institutions for the evaluation of swallowing problems. The multidisciplinary approach outlined will permit a better understanding and more accurate diagnosis of the functional or organic lesions affecting the swallowing mechanisms. Illustrative cases are presented.