Low bone mass in patients with motor disability: prevalence and risk factors in 59 Finnish children.

AIM: Children with motor disabilities are at increased risk of compromised bone health. This study evaluated prevalence and risk factors of low bone mass and fractures in these children. METHOD: This cross-sectional cohort study evaluated bone health in 59 children (38 males, 21 females; median age 10 y 11 mo) with motor disability (Gross Motor Function Classification System levels II-V). Bone mineral density (BMD) in the lumbar spine was measured with dual-energy X-ray absorptiometry; BMD values were corrected for bone size (bone mineral apparent density [BMAD]) and skeletal maturity, and compared with normative data. Spinal radiographs were obtained to assess vertebral morphology. Blood biochemistry included vitamin D concentration and other parameters of calcium homeostasis. RESULTS: Ten children (17%) had sustained in total 14 peripheral fractures; lower-limb fractures predominated. Compression fractures were present in 25%. The median spinal BMAD z-score was -1.0 (range -5.0 to 2.0); it was -0.6 in those without fractures and -1.7 in those with fractures (p=0.004). Vitamin D insufficiency was present in 59% of participants (serum 25-hydroxyvitamin D <50 nmol/l) and hypercalciuria in 27%. Low BMAD z-score and hypercalciuria were independent predictors for fractures. INTERPRETATION: Children with motor disability are at high risk of peripheral and vertebral fractures and low BMD. Evaluation of bone health and prevention of osteoporosis should be included in the follow-up.

Bone mineral density and sex hormone status in intellectually disabled women on progestin-induced amenorrhea.

OBJECTIVE: To evaluate bone mineral density (BMD) and hormonal status in female patients with intellectual disability and a history of progestin-induced amenorrhea. DESIGN: Cross-sectional study. SETTING: Nursing home. SAMPLE: The study included 51 patients with a history of therapeutic amenorrhea (age 23-77 years, mean 45 years); 115 staff members (age 21-64 years, mean 45 years) at the same nursing homes served as controls. METHODS: Calcaneal BMD was measured for all (Peripheral Instantaneous X-ray Imaging Lunar Bone Densitometer); blood samples for serum levels of estradiol (E(2)), follicle stimulation hormone (FSH) and lutenizing hormone (LH) were obtained only for the patients. RESULTS: The patients showed significantly lower age and weight-adjusted BMD than the controls (0.35 g/cm(2)+/-0.13 vs. 0.53 g/cm(2)+/-0.09, p<0.001). BMD values did not differ between pre- (N=29) and postmenopausal (N=22) patients. Osteoporosis was observed in 57% of the patients and only in 2% of the controls. Four patients (8%) but none of the controls had sustained a bone fracture during the preceding five years. Most premenopausal patients had hypogonadotropic hypogonadism, as shown by low serum E(2), LH and FSH levels in 83%, 69%, and 59% of the cases. Postmenopausal patients showed normal hormonal status for their age. CONCLUSION: Osteoporosis with concomitant fractures is prevalent in women with intellectual disability on therapeutic amenorrhea. Progestin-induced amenorrhea results in hypogonadism, an established risk factor for osteoporosis. New strategies for the management of menstruation should be considered.

Idiopathic generalised epilepsies with 3 Hz and faster spike wave discharges: a population-based study with evaluation and long-term follow-up in 71 patients.

For several years we have been following patients with intractable, childhood-onset idiopathic generalised epilepsies with > or = 3 Hz spike-wave discharges. Our need to find explanations for their intractability was the starting point for this study. We were interested in identifying characteristics, which would predict intractability; evaluating how these patients were treated and whether polytherapy was useful. We identified patients with > or = 3 Hz spike-wave discharges by reviewing EEG reports recorded between 1983 and 1992. Data were collected from medical records and through personal interviews. We identified 82 patients with tentative idiopathic generalised epilepsy. Eleven were excluded. Thirty-eight patients had childhood absence epilepsy, 18 had juvenile absence epilepsy, 13 had juvenile myoclonic epilepsy and two had eyelid myoclonia with absences: 89.5, 78, 38 and 0% of the patients in each group, respectively, had been seizure free for more than 2 years. Twenty percent of the patients had intractable seizures. All intractable patients with juvenile absence epilepsy had rhythmic, random eyelid blinking and generalised tonic-clonic seizures. A history of more than ten generalised tonic-clonic seizures was associated with intractability in juvenile myoclonic patients. Monotherapy with ethosuximide or valproate resulted in seizure control in 65% of patients. Seventeen patients (24%) were treated with polytherapy, six achieved remission. These six patients had childhood absence epilepsy and juvenile absence epilepsy. Positive outcome was found in childhood absence epilepsy and juvenile absence epilepsy. Intractable seizures were more frequent among patients with juvenile myoclonic epilepsy. None of them benefited from polytherapy with conventional anti-epileptic drugs.