Jerry W. Swanson

Neurologic Manifestations of Churg-Strauss Syndrome

OBJECTIVE To determine the frequency and the types of neurologic involvement in a series of patients with Churg-Strauss syndrome (CSS). DESIGN We reviewed the medical records of 47 consecutive patients with CSS who were examined at the Mayo Clinic between January 1974 and June 1992. MATERIAL AND METHODS The study patients were classified into two groups: (1) those with a histopathologically confirmed diagnosis of CSS who had evidence of either vasculitis or Churg-Strauss granuloma, the presence of asthma, and peripheral eosinophilia (more than 10% eosinophils) on at least one differential leukocyte count (N = 33) and (2) those with a clinical diagnosis of CSS who had evidence of vasculitis based on either multiple mononeuropathy or necrotizing cutaneous lesions, the presence of asthma, and peripheral eosinophilia (more than 10% eosinophils) on at least one differential leukocyte count (N = 14). RESULTS Of the 47 patients, 29 (62%) had neurologic involvement. Peripheral neuropathy was detected in 25 patients: 17 had multiple mononeuropathy, 7 had distal symmetric polyneuropathy, and 1 had an asymmetric polyneuropathy. Three patients had cerebral infarctions. Less commonly identified problems included radiculopathies, ischemic optic neuropathy, and bilateral trigeminal neuropathy. Asthma preceded the onset of neurologic involvement in all cases (mean duration, 6.7 years. Follow-up data, when available, showed that corticosteroid therapy usually yielded improvement or stabilization. CONCLUSION Neurologic involvement is common in CSS, usually manifesting as peripheral neuropathy. In this series of patients, asthma preceded the neurologic manifestations.

Stereotactic radiosurgery using the gamma knife for acoustic neuromas

PURPOSE To assess the efficacy and toxicity of stereotactic radiosurgery using the gamma knife for acoustic neuromas. METHODS AND MATERIALS Between January 1990 and January 1993, 36 patients with acoustic neuromas were treated with stereotactic radiosurgery using the gamma knife. The median maximum tumor diameter was 21 mm (range: 6-32 mm). Tumor volumes encompassed within the prescribed isodose line varied from 266 to 8,667 mm3 (median: 3,135 mm3). Tumors < or = 20 mm in maximum diameter received a dose of 20 Gy to the margin, tumors between 21 and 30 mm received 18 Gy, and tumors > 30 mm received 16 Gy. The dose was prescribed to the 50% isodose line in 31 patients and to the 45%, 55%, 60%, 70%, and 80% isodose line in one patient each. The median number of isocenters per tumor was 5 (range: 1-12). RESULTS At a median follow-up of 16 months (range: 2.5-36 months), all patients were alive. Thirty-five patients had follow-up imaging studies. Nine tumors (26%) were smaller, and 26 tumors (74%) were unchanged. No tumor had progressed. The 1- and 2-year actuarial incidences of facial neuropathy were 52.2% and 66.5%, respectively. The 1- and 2-year actuarial incidences of trigeminal neuropathy were 33.7% and 58.9%, respectively. The 1- and 2-year actuarial incidence of facial or trigeminal neuropathy (or both) was 60.8% and 81.7%, respectively. Multivariate analysis revealed that the following were associated with the time of onset or worsening of facial weakness or trigeminal neuropathy: (a) patients < age 65 years, (b) dose to the tumor margin, (c) maximum tumor diameter > or = 21 mm, (d) use of the 18 mm collimator, and (e) use of > five isocenters. The 1- and 2-year actuarial rates of preservation of useful hearing (Gardner-Robertson class I or II) were 100% and 41.7% +/- 17.3, respectively. CONCLUSION Stereotactic radiosurgery using the gamma knife provides short-term control of acoustic neuromas when a dose of 16 to 20 Gy to the tumor margin is used. Preservation of useful hearing can be accomplished in a significant proportion of patients.

Incidence of migraine headache A population‐based study in Olmsted County, Minnesota

To determine the incidence of clinically detected migraine headache in the defined population of Olmsted County, MN, 1979-1981, we screened over 6,400 patient records from several diagnostic rubrics using the unique resources of the Rochester Epidemiology Project for population-based studies. We identified 629 Olmsted County residents who fulfilled the International Headache Societys 1988 criteria for newly diagnosed migraine headache between 1979 and 1981. The overall age-adjusted incidence was 137 per 100,000 person-years for males and 294 per 100,000 person-years for females. The highest incidence in females was among those aged 20 to 24 years (689 per 100,000 person-years), and in males, the highest incidence was among those aged 10 to 14 years (246 per 100,000 person-years). From 1979 to 1981, there was a striking increase in the age-adjusted incidence in those under 45 years of age: the incidence increased 34% in women and 100% for men. This is the first population-based study of migraine incidence across all ages.

Incidence of cluster headaches A population‐based study in Olmsted County, Minnesota

The epidemiology of cluster headache is virtually unknown. Using the unique resources of the Rochester Epidemiology Project for population-based studies, we identified 26 Olmsted County, Minnesota, residents who fulfilled the International Headache Society criteria for newly diagnosed cluster headache between 1979 and 1981. As part of a large study of migraine and cluster headache, we screened more than 6,400 patient records from several diagnostic rubrics to obtain this cohort, accomplished case ascertainment exclusively through medical record review, and assigned diagnoses by the consensus of two neurologists. The age-adjusted incidence was 15.6 per 100,000 person-years (p-y) (95% CI, 8.9 to 22.3) for males and 4.0 per 100,000 p-y for females (95% CI, 0.4 to 7.6). The overall age- and sex-adjusted incidence was 9.8 per 100,000 p-y (95% CI, 6.0 to 13.6) or approximately 1/25 that of migraine. The peak incidence was among men aged 40 to 49 years and women 60 to 69 years. There was a higher than expected prevalence of history of smoking among males with cluster headaches (p< 0.05), supporting the possibility that smoking predisposes to the development of cluster headaches in men

A migrainous syndrome with cerebrospinal fluid pleocytosis

Abnormalities of cerebrospinal fluid (CSF) are rare in migraine, even when accompanied by focal neurologic signs and symptoms. Seven patients without a prior history of migraine experienced a limited series of migraine-like attacks associated with abnormalities of the CSF. The episodic, severe headaches were preceded and accompanied by sensory, motor, speech, and visual disturbances. The CSF showed a predominantly lymphocytic pleocytosis, increased protein, and elevated opening pressure. All seven patients recovered completely. We postulate that the migrainous episodes were symptomatic of an underlying inflammatory disorder of the central nervous system. This distinctive combination of symptoms and CSF findings constitutes a benign syndrome.

Autonomic dysfunction in migraineurs.

Objective.—To evaluate autonomic function and sympathovagal balance in migraineurs and healthy controls.

Increasing incidence of medically recognized migraine headache in a United States population

OBJECTIVE To investigate trends in the incidence of medically recognized migraine in Olmsted County, Minnesota over approximately a decade. METHODS The authors used the records-linkage system of the Rochester Epidemiology Project to identify individuals whose records included any diagnostic rubric related to headache for the 3-year period 1979 through 1981 and the 2-year period 1989 through 1990. A nurse abstracter and a neurologist (J.W.S.) reviewed the complete history of each potential case and assigned a diagnosis using the International Headache Society classification (IHS, modified). Only patients who consulted a doctor for their headache and had their initial visit for migraine within the study years were considered as incident cases. RESULTS The incidence of medically recognized migraine increased in female subjects between the 1979-through-1981 period and the 1989-through-1990 period for all ages, but particularly among those who were aged 10 to 49 years. The peak incidence rate at age 20 to 29 years increased from 634.5 new cases per 100,000 person-years in 1979 through 1981 to 986.4 in the 1989-through-1990 period (absolute increase 351.9; relative increase 56%). The rise in incidence in female subjects was most sizable for migrainous disorder (IHS code 1.7); smaller increases were noted for migraine without aura and with typical aura. Only a slight absolute increase in migraine incidence rates was observed in male subjects, restricted to those 10 to 19 years of age (absolute increase 174.7; relative increase 89%). CONCLUSIONS Although the incidence rates reported here are restricted to patients who consulted a doctor for their headache, the authors suggest that the incidence of migraine has increased over time in female subjects, especially those of reproductive age. The increase was most pronounced for migrainous disorder. Incidence rates were more stable in male subjects over time.

Chronic paroxysmal hemicrania presenting as otalgia with a sensation of external acoustic meatus obstruction: two cases and a pathophysiologic hypothesis.

Objective.—To describe two cases of chronic paroxysmal hemicrania manifested by otalgia with a sensation of external acoustic meatus obstruction and to suggest that the trigeminal‐autonomic reflex is a mechanism for the sensation of ear blockage.

The ‘red Ear Syndrome’ Revisited: Two Cases and a Review of Literature

The first description of what subsequently came to be known as the red ear syndrome (RES) was by Lance in 1994 (1) (Table 1, cases 2-4). Subsequently he reported a total of 12 patients with what he called the red ear syndrome (2) (Table 1). The syndrome was characterized by attacks of unilateral ear discomfort or burning during which the ear became red. The discomfort often extended beyond the ear. He reported an association with upper cervical disorders, glossopharyngeal and trigeminal neuralgia, temporomandibular joint (TMJ) dysfunction, and a thalamic syndrome. In two of the 12 cases (Table 1, cases 11 and 12) no cause was apparent. In some cases the episodes were spontaneous and in others they were precipitated by touch, exertion, heat or cold, neck movements, stress, cleaning the ear, washing hair or brushing it the wrong way, and eating or drinking. While some of their patients had a history of headaches (including migraines), a consistent relationship to migraine headaches was not identified.

Headache after resection of acoustic neuroma

Long‐lasting severe headaches are reported to occur in up to 83% of patients who have undergone resection of acoustic neuroma, especially through a suboccipital approach. These headaches, however, are not well defined. The objective of this study was to assess the frequency and character of new‐onset headaches after resection of acoustic neuroma by a suboccipital approach with cranioplasty. Review of the medical record was followed by a telephone interview with 48 patients (67% female; mean age, 52 years) who had undergone resection of an acoustic neuroma through a suboccipital craniotomy during the 2 years before the study. Of the 48 patients, 58% had post‐operative head pain that lasted more than 7 days and could be categorized into two types. A moderate to severe, short‐term head pain with gradual resolution occurred in 35% of the patients, and a mild, unremitting pain was reported by 23%. Both types of pain had a dull ache or pressure quality and were adjacent to or confined to the incisional area. Overall, 77% of the patients were pain‐free within 4 months after operation. Age, sex, tumor size, or preoperative history of headache did not influence development of the postoperative pain.