Minimum incidence of primary cervical dystonia in a multiethnic health care population.

BACKGROUND: The two existing estimates of the incidence of primary cervical dystonia were based on observations in relatively ethnically homogeneous populations of European descent. OBJECTIVE: To estimate the minimum incidence of primary cervical dystonia in the multiethnic membership of a health maintenance organization in Northern California. METHODS: Using a combination of electronic medical records followed by medical chart reviews, we identified incident cases of cervical dystonia first diagnosed between 1997 and 1999. RESULTS: We identified 66 incident cases of cervical dystonia from 8.2 million person-years of observation. The minimum estimate of the incidence of cervical dystonia in this population is 0.80 per 100,000 person-years. Ethnicity-specific incidence rates were calculated for individuals over age 30. Incidence was higher in white individuals (1.23 per 100,000 person-years) than in persons of other races (0.15 per 100,000 person-years, p < 0.0001). The minimum estimated incidence was 2.5 times higher in women than in men (1.14 vs 0.45 per 100,000 person-years, p = 0.0005). The average age at diagnosis was higher in women (56 years) than in men (45 years, p = 0.0004). There was no significant difference in reported symptom duration prior to diagnosis between women and men (3.9 vs 5.3 years). CONCLUSION: The estimated incidence of diagnosed cervical dystonia among white individuals in this Northern Californian population is similar to previous estimates in more ethnically homogeneous populations of largely European descent. The incidence in other races, including Hispanic, Asian, and black appears to be significantly lower. The incidence is also higher in women than in men.

Effect of reproductive factors and postmenopausal hormone use on the risk of Parkinson disease.

OBJECTIVE: Parkinson disease (PD) is less common in women possibly because of hormonal or reproductive influences. The objective of this study was to evaluate the associations of reproductive factors and postmenopausal hormone use with the risk of PD among postmenopausal women. METHODS: Incident cases (n = 178) and randomly selected age-matched controls (n = 189) who were members of the Kaiser Permanente Medical Care Program (KPMCP) of Northern California participated in the study conducted during the years 1994 to 1995. Statistical analyses were carried out using logistic regression. RESULTS: The association of postmenopausal hormone use with PD risk depended on the type of menopause. Among women with history of a hysterectomy with or without an oophorectomy, estrogen use alone was associated with a 2.6-fold increased risk (adjusted odds ratio (OR) 2.6, 95% CI: 1.1 to 6.1) and significant trends in the risk of PD were observed with increasing duration of estrogen use, but disease risk was not influenced by recency of use. In contrast, among women with natural menopause, no increased risk of PD was observed with hormone use (estrogen alone or a combined estrogen-progestin regimen). Early age at final menstrual period (44 years or younger) was associated with reduction in risk (adjusted OR 0.5, 95% CI: 0.3 to 1.0). Age at menarche and parity were not associated with the risk of PD. CONCLUSION: Postmenopausal use of estrogen alone may increase the risk of Parkinson disease (PD) among women with a hysterectomy. Among women with natural menopause for whom the usual treatment is combined estrogen-progestin therapy, no increased risk of PD was observed.

Persistent pulmonary hypertension in a neonate with cystic adenomatoid malformation of the lung following lobectomy: survival with prolonged extracorporeal membrane oxygenation therapy.

A full-term neonate is reported with congenital cystic adenomatoid malformation of the lung treated by lobectomy with development of pulmonary hypertension. The infant was successfully treated with extracorporeal membrane oxygenation (ECMO) for persistent pulmonary hypertension, which developed postoperatively. An 18-day course of venovenous ECMO was necessary to effectively reverse the severe pulmonary hypertension. This was probably a result of significant pulmonary hypoplasia of the compressed lung. Although not all congenital cystic adenomatoid malformations of the lung are associated with pulmonary hypoplasia and persistent pulmonary hypertension, this is one case where severe pulmonary hypertension developed secondary to a mass effect by a large lesion in the chest.

Elder hearing aids. Infrared listening device in a geriatric day center.

1. An infrared listening device (IRLD) is a device that changes an auditory signal into infrared light. This signal is delivered to an infrared receiver via light-emitting diodes. The signal is then converted back into auditory energy. 2. Nineteen percent of elders at the Day Care Center for the Elderly who participated in the survey expressed an interest in using an IRLD if it were made available to them. More than half of the clients in this group were successful in using the listening device. 3. The application of an IRLD can add a dimension of independence to the lives of older adults. For those individuals who are unable to operate a conventional hearing aid, an IRLD can offer the benefits of amplification in a form that may be easier to comprehend and operate.

Brief communication: effect of pharmacologic doses of vitamin B6 on carpal tunnel syndrome, electroencephalographic results, and pain.

The role of vitamin B6 as a therapeutic agent in the treatment of carpal tunnel syndrome was examined by monitoring both the standard clinical and electrophysiological parameters for entrapment neuropathy at the wrist. Electroencephalogram (EEG) studies were done in an attempt to identify patients most likely to benefit from B6 treatment. EEGs did not prove useful as predictors of clinical response to vitamin B6. Our patients, however, did not show any abnormalities prior to treatment, and no changes occurred during the treatment period. Motor latency, while the most common screening test for carpal tunnel syndrome, was not significantly changed during the course of treatment. It did not prove to be a useful test for monitoring clinical effectiveness of the treatment. Parameters showing the greatest changes were pain scores and sensory latency, which most closely paralleled clinical assessments. Pain scores, more than any other parameters, were improved in these patients following vitamin B6 treatment. Vitamin B6 has been shown to change pain thresholds in clinical and laboratory studies. This may be the basis of the significant improvement in pain scores when electrophysiologic data showed only mild improvement. This study suggests that vitamin B6 deficiency may not be a cause of carpal tunnel syndrome in spite of the observed therapeutic effect, without toxicity, of vitamin B6 treatment.

Vitamin B6 in clinical neurology.

Many conditions in clinical neurology may be responsive to pyridoxine as a therapeutic agent. The current difficulty is in trying to isolate the conditions that are most likely to respond. Treating seizures is a major part of a neurologic practice. Our current therapeutic agents are only partially successful and limited by multiple side effects. One problem is that patients often have to take these agents for an entire lifetime, further raising the risk of toxicity. If pyridoxine supplementation can improve the efficacy of currently used medications, it will be gladly accepted into our therapeutic arsenal. Headache, chronic pain, and depression all appear to run together in many of our patients. The observations that serotonin deficiency is a common thread between them and that pyridoxine can raise serotonin levels open a wide range of therapeutic options. Small studies have been carried out with mixed success. Comparison with amitriptyline in the treatment of headache appears to show about equal efficacy, although side effects would be expected to be more of a problem with the amitriptyline. Behavioral disorders are relatively common and continue to be a major problem, disrupting the lives of the patients and their families. Current treatments are not acceptable to most people because of the risk of side effects with long-term usage. If, as Dr. Feingold suggests, many of these problems are caused by "toxic" exposures to chemicals that are pyridoxine antagonists, supplementation at early ages may reduce the incidence of hyperactivity and aggressive behavior. This raises the question of safety. Is pyridoxine safe for long-term use in large segments of the population, including children? The studies on children with Down's syndrome and autism, utilizing much higher doses than are used for other therapeutic purposes, seem to indicate relative safety if carefully monitored. Studies involving large population groups with carpal tunnel syndrome, all adults, using 100-150 mg/day have shown minimal or no toxicity in five- to 10-year studies. Women self-medicating for PMS taking 500 to 5000 mg/day have shown peripheral neuropathy within one to three years. It would appear from this retrospective analysis that pyridoxine is safe at doses of 100 mg/day or less in adults. In children there is not enough data to make any sort of suggestion. Because the major neurologic complication is a peripheral neuropathy and the causes of this condition are myriad, pyridoxine may cause neuropathy only in patients with a pre-existing susceptibility to this condition.

Tricorrectional bunionectomy with AO screw fixation.

A 16-month preliminary study was performed on 58 patients for corrective surgery of hallux abducto valgus, with AO4 screw fixation. The procedure is a modification of the bi-plane Austin procedure, called a tricorrectional bunionectomy. The authors present the procedure with objective and subjective findings. The principles of bone healing and internal fixation using AO screw fixation are discussed.

Loss of fluorescence by anthracycline antibiotics: effects of xanthine oxidase and identification of the nonfluorescent metabolites.

Rat liver cytosol and buttermilk xanthine oxidase both converted 7-deoxypyrromycinone, the 7-deoxyaglycone of marcellomycin, a new anthracycline antibiotic, to a nonfluorescent compound under anaerobic conditions and in the presence of an electron donor. Reduced nicotinamide adenine dinucleotide and reduced nicotinamide adenine dinucleotide phosphate were equally effective electron donors for liver cytosol, and xanthine was the best cofactor for xanthine oxidase. However, xanthine was inactive with liver cytosol. Reactions with xanthine oxidase obeyed Menten-Michaelis kinetics and were inhibited by allopurinol. No xanthine oxidase activity was detected in liver cytosol. Xanthine oxidase also induced a loss of fluorescence when incubated with 7-deoxydaunorubicin aglycone. The nonfluorescent metabolite of 7-deoxypyrromycinone was tentatively identified as the dihydroquinonic derivative of the parent deoxyaglycone on the basis of its spectrophotometric, fluorescent, thin layer chromatographic, and mass spectral characteristics. Our data demonstrate that more than one enzymatic activity, xanthine oxidase, and an unidentified rat liver cytosolic enzyme convert the 7-deoxyaglycones of anthracycline antibiotics to nonfluorescent metabolites.

Neurologic examination in children.

The neurologic examination in children requires an ability to communicate with the parents and child, a knowledge of developmental milestones, and an index of suspicion that encompasses multiple physiologic systems. Observation is the single most important skill. When coupled with the background knowledge to put these observations into their appropriate places, the diagnostic considerations can be dramatically reduced. The use of specialized testing may be minimized by a thorough history, physical examination, and review of prior evaluations. Most neurologic conditions have a slow course. If the diagnosis is not readily defined, a series of follow-up visits may give a clearer picture of the underlying process. Children are constantly changing. The examinations we perform must be infinitely flexible to accommodate this fact.

Cyst and cystlike lesions of the foot.

A variety of cyst and cystlike lesions may present in the osseous structures of the foot. Such lesions are infrequent in their occurrence, and often cannot be diagnosed on the basis of radiographic appearance. In addition to the morphologic data that can be derived from evaluation of standard radiographs, other diagnostic radiographic studies such as tomography, CT scanning, radionuclide bone imaging, angiography, or other studies may be required to ascertain the nature and extent of cystlike lesions of the foot. This is dependent on histopathologic information derived from biopsy. A variety of cyst and cystlike lesions of the foot are presented with a review of their basic morphology and histopathologic, clinical, and prognostic characteristics.

Sonography of lung carcinoma metastatic to the kidney.

Metastasis of primary lung cancer to the kidney is commonly discovered at autopsy, but the diagnosis is rarely made antemortem. Four cases are reported with ultrasound findings and the literature on the subject is reviewed.