X-ray CT of Duchenne muscular dystrophy skeletal muscles--chronological study for five years]

To clarify when and how rapidly individual muscles are damaged in the course of Duchenne muscular dystrophy (DMD), we followed X-ray CT of whole body skeletal muscles from 29 cases of DMD patients (age ranging 3 to 23 years) for a period of 2 to 6 years. Each patient had 2 to 5 scans with an average of 3.3. We evaluated chronological changes of 23 muscles from the entire body using the muscle damage stage defined as follows. The stages were classified into the following 5 stages: stage 0; normal, 1; area of fatty replacement less than 10% of whole muscle area, 2; area of fatty replacement between 10 to 50%, 3; area of fatty replacement 50 to 90%, 4; almost complete fatty replacement. Each muscle had its own period of rapid degeneration starting at 5 to 10 years of age and, continuing 5 to 10 years. In some muscles such as gluteus maximus or quadriceps femoris, fatty replacement started at 5 years or earlier and progressed for five years, while other muscles such as splenius capitis, damage started much later, e.g. around 10 years of age and the progression was much slower. There was a variation of at least 5 years among individual patients in any muscle damage stage, reflecting the variability of clinical severity in each patient. On the basis of the above results we defined the whole body muscle damage index as a summation of the muscle damage stages of the following five muscles: gluteus maximus, quadriceps femoris, gracilis, medial head of gastrocnemius and splenius capitis.(ABSTRACT TRUNCATED AT 250 WORDS)     

Plasma levels and membrane transports in red blood cell of tyrosine and tryptophane in depression. Evaluation at baseline and recovery

The erythrocyte membrane transports (MT) of L-tyrosine and L-tryptophan, as well as their plasma levels, were measured in 143 depressed patients after a wash-out period of one week and before any antidepressant treatment, and then at regular intervals until they recovered. 49 normal subjects served as a control group. The perturbations of these biologic variables at baseline were different according to diagnostic groups (DSM III), compared to controls: significant decrease of the two MT in depressed bipolar disorders; significant decrease of MT in depressed bipolar disorders; significant decrease of MT tyrosine and significant increase of MT tryptophan in recurrent major depressions; no significant changes of MT in dysthymic disorders. The plasma levels of tyrosine and total tryptophan show a significant diminution in the 3 groups. The study of the evolution of these same variables after antidepressant treatment show a return to normal of the MT at recovery, as well as the plasma levels of tyrosine. The plasma levels of tryptophan stay low in depressed bipolar disorders and recurrent major depressions.     

A survey of hospitals in New York State treating psychiatric patients with chemical abuse disorders.

Eighty-eight of 143 hospitals in New York State providing psychiatric inpatient treatment responded to a mailed questionnaire designed to determine the size of three subgroups of chemical abusers--alcohol abusers, drug abusers, and polychemical abusers--among inpatients with psychiatric diagnoses, as well as the availability of services for these patients. Data for New York City and its metropolitan area were analyzed separately. In 1987 almost one-third of psychiatric admissions both in and outside the metropolitan area had comorbid chemical abuse disorders. Seventy-five percent of patients in the metropolitan area with comorbid chemical abuse had a drug abuse disorder; in rural areas 88 percent of patients with chemical abuse disorders abused alcohol. Both hospital- and community-based aftercare services, especially in the metropolitan area, were less available to psychiatric patients with chemical abuse than to patients without these disorders.     

Altered acceptability of and preference for sugar solutions by diabetic rats is normalized by high-fat diet

Two experiments examined the ingestive responses of streptozotocin-diabetic rats fed low-fat or high-fat diets to glucose, fructose, sucrose and maltose solutions in brief (30 min) intake tests. In Experiment 1, one-bottle acceptability tests were used whereas two-bottle preference tests were used in Experiment 2. Three main findings resulted from these studies. Firstly, diabetic rats fed the low-fat diet displayed a reduced acceptance of and preference for all concentrated sugar solutions. Secondly, glucose consumption patterns of diabetic rats fed the low-fat diet were distinctly different from their responses to the other sugars. Thirdly feeding high-fat diets, either high or low in carbohydrate, normalized the responses of diabetic rats to the sugar solutions. The results suggest that feeding high-fat diets to diabetic rats normalizes their responses to sugar solutions because of reductions in hunger and thirst associated with the provision of a utilizable source of calories and an improvement in body fluid balance.     

Pedaling automatism in atypical West syndrome: a case report]

A seven-month-old girl with atypical West syndrome with pedaling automatism was reported. She started to have early infantile epileptic encephalopathy with suppression-burst (EIEE) at age 14 days followed by infantile spasms at 3 months of age. She began to have spasms with automatism at 4 month, mainly consisting of pedaling movements of both feet associated with tonic spasms of upper extremities and writhing of trunk. Ictal EEG showed irregular slow waves with occasional spikes predominantly over the right hemisphere mixed with artifacts. Interictal EEG during sleep showed suppression-burst patterns. Cerebral atrophy and microcephaly were revealed on brain CT, immature myelination on MRI and decreased blood flow in the frontal, temporal, and parietal lobes on the right on PET scan. Pedaling automatism is rare in infancy and its clinical significance was discussed in relation to West syndrome and complex partial seizures.     

Three-dimensional human pattern visual evoked potentials. II. Multiple sclerosis patients.

Pattern visual evoked potentials were obtained from 46 patients with definite relapsing/remitting multiple sclerosis, using both a conventional 5-channel occipital array and a 3-D recording technique consisting of three bipolar derivations approximating the three dimensions of space. These three orthogonal wave forms were displayed as a 3-D Lissajous trajectory for each subject. Two of the 15 patients with completely normal conventional pattern VEPs had abnormalities of the orientation of the B-C curvilinear segment of the 3-D pattern VEPs. Delays in the first major occipital positive component (P100) were evident using both techniques; the correlation between P100 latency and the latency of the corresponding trajectory apex was r = 0.99 (P less than 0.01). Post-chiasmal MRI abnormalities were associated with 3-D VEP orientation abnormalities. Three-dimensional pattern VEPs are moderately more sensitive than conventional pattern VEPs at detecting dysfunction posterior to the optic chiasm in demyelinating disease and do not require the use of eccentric fixation to do so.     

Unusual case of Klüver-Bucy syndrome in a Chinese boy.

Following an encephalopathic illness, a 13-year-old Chinese boy had a partial form of Klüver-Bucy syndrome with emotional disturbance, recent memory loss, hypersexuality, and polyphagia. Other unusual features included narcolepsy, polydipsia, and polyuria. Virologic studies failed to incriminate the etiologic agent, including herpes simplex virus. Brain biopsy of the frontal lobe demonstrated Alzheimer type II astrocytosis.