[A case of Hallervorden-Spatz syndrome with marked atrophy of the brainstem and cerebellum].

We report a 20-year-old female with Hallervorden-Spatz syndrome (HSS). This patient is the product of consanguineous parents. She developed genu valgum, tendency to fall and mental deterioration at the age of 6, decrease of the number of spoken words at the age of 14, dysarthria, unsteady gait, postural tremor of the upper extremities, dystonic posture of hands and double incontinence at the age of 16. Her disease progressed slowly. Neurological examination on admission revealed severe mental retardation, optic atrophy, forced grasping, hyperactive tendon reflexes in the upper extremities and bilateral Babinski sign. An extensive laboratory investigation including the leukocyte lysosomal enzymes, serum amino acid analyses, copper studies and ceruloplasmin were almost within normal limits. MRI, T2 weighted images, showed markedly decreased signal intensity in the globus pallidus but substantia nigra and increased signal intensity in diffuse cerebral white matter. T1 weighted images showed marked atrophy of the brainstem and cerebellum. She met the diagnostic criteria for HSS by Swaiman; we diagnosed her as HSS group II. HSS is characterized by the presence of many spheroids in the central nervous system which is similar to neuroaxonal dystrophy (ND). However, clinical and pathological differences exist between HSS and ND, the precise classification of the two conditions has remained controversial. Although there are many reported cases in which both conditions overlap, this is the first reported case that simultaneously demonstrates increased iron deposition in the globus pallidus, marked atrophy of the brainstem and cerebellum and typical clinical course compatible with HSS.

[Treatment of threatened premature labor due to subclinical intrauterine infection].

Recent reports reveal that some threatened premature labors (tpl) are caused by intrauterine infections. And the prognosis of tpl due to intrauterine infections are worse than tpl due to other factors because of lower sensitivity to the beta-stimulants. So we examined the relationship between tpl and intrauterine infections retrospectively by obtaining intracervical cultures of inpatients who suffered from tpl, and prospectively by getting intracervical cultures from healthy pregnant women at a gestational age around 20 weeks, and divided them into two groups--the infectious group and non-infectious group--and studied the tpl rate. The patients who had positive bacterial cervical cultures were again divided into two groups. One group had antibiotics administered orally and the other had none. The tpl rate was observed. The results are: 1. Thirty two point six percent of tpl inpatients were found to be infected intracervically. No statistical significance was noted in the microbodies detected. 2. Three cases of premature labor at less than 35 gestational weeks were observed in the infected group. 3. The majority of infected group showed high CRP levels, and the study of Preterm Labor Score (PLS) showed that most cases over 4 points of PLS were infected. 4. In a prospective study, 18 of 100 healthy pregnant women showed positive intracervical bacterial cultures. The tpl rates were 44.4% in all of the infected group. Twenty two point two percent of tpl rate in the antibiotic administration group and 66.7% in the non-administration group (p less than 0.01). And the non-infected tpl rate was 11.0% (p less than 0.01), or only 9 cases out of 82.(ABSTRACT TRUNCATED AT 250 WORDS)