Multiple, primary spinal-paraspinal hydatid cysts.

A patient is presented with widespread primary hydatid cysts in spinal-paraspinal locations, secondary to Echinococcus granulosus. An alternative mechanism to explain how the embryos gained access to the body is proposed: The embryos penetrated the intestinal muscle and may have directly entered into the inferior vena cava system through small venous connections between this system and portal circulation. Various conditions in daily life associated with Valsalva maneuver might have caused such an atypical passage of the embryos to the inferior caval system toward the retroperitoneum and spinal-paraspinal structures via lumber epidural venous plexuses.

Familial brachyolmia.

Brachyolmia is characterized clinically by short stature and radiographically by generalized platyspondyly without significant long bone abnormalities. A healthy 13-(8/12) year-old girl was referred for evaluation of short stature. The parents were first cousins. She had two older brothers and a younger brother and sister. On examination, her height was 116.2 cm, height SDS -6.2, armspan 135 cm. She had completed puberty. Except for her short trunk and lower extremities and mild scoliosis, she appeared normal. At 12 years old, the younger brother had short stature. His height was 104.7 cm, height SDS -6.2, armspan 116 cm. The younger sister was 3 years old. Her height was 84.2 cm, height SDS -2.9, armspan 85 cm. Other findings were normal in the younger sister and brother. The other members of the family were of normal stature and appearance. The proband's growth hormone stimulation tests, thyroid function tests, sex steroids, gonadotropins and blood biochemistry were found normal. There were similar radiological findings in the three siblings. There was platyspondyly, narrowing of intervertebral spaces in all vertebral bodies. The iliac bones were broad. No metaphyseal irregularity and normal epiphyses were detected in all patients. No significant changes were seen in long bones and skull. According to the physical and radiological findings, the patients were evaluated as brachyolmia.

Neuropsychologic sequelae in the long-term survivors of childhood acute lymphoblastic leukemia.

The neurotoxicity of either systemic chemotherapy or central nervous system prophylaxis was studied in 19 children treated for acute lymphoblastic leukemia (ALL). They had completed ALL therapy at least a year before and survived more than 5 years after diagnosis. The duration between age at diagnosis and age at investigation was 8.6 +/- 2.7 years (5-15 years). Neuropsychologic tests, cranial magnetic resonance imaging (MRI), and evoked potentials (EP) were studied. Seventeen healthy siblings were taken as a control group. Emotional evaluation was done using the childhood depression inventory and Beck depression inventory. Cognitive functions were evaluated using Wechsler's Intelligence Scale for Children-Revised (WISC-R) or the Wechsler's Adult Intelligence Scale-Revised (WAIS-R) tests, which were adapted to Turkish children. Performance and total IQ scores (94.0 +/- 16.8 and 92.2 +/- 16.5) were significantly low as compared to the control group (112.1 +/- 18.9 and 105.4 +/- 14.2) (p = .007 and p = .02). Abnormal MRI findings were found in 33.3% (6/18). Three out of 18 patients (16.6%) had abnormal auditory while 5 out of 17 patients (29.5%) displayed abnormal visual EPs. Abnormal findings in MRI, cognitive examination, and electrophysiologic testing were not associated with age at diagnosis, radiotherapy doses, intermediate/high-dose systemic methotrexate administration or central nervous system involvement. But more patients must be studied to demonstrate discrete outcomes of neurotoxicity in long-term survivors of childhood leukemia.

Progressive pseudorheumatoid arthropathy of childhood.

Progressive pseudorheumatoid arthropathy of childhood (PPAC) described by Spranger et al is a rare autosomal recessive disorder. An 11 year-old girl was diagnosed as having PPAC at Ege University, Faculty of Medicine, Department of Paediatrics. Her complaints of painful joints, difficulty in walking and joint contractures began at the age of 3 years and she was treated for juvenile rheumatoid arthritis for 8 years. Her symptoms did not respond to nonsteroid anti-inflammatory treatment. During her last hospitalisation period, she was reinvestigated. Radiological examination showed spondyloepiphyseal dysplasia, severe acetabular irregularity and osteoporosis. All the laboratory test results for rheumatoid arthritis were negative. The clinical and radiological findings of the patient are illustrated.

Associated brain abnormalities in patients with corpus callosum anomalies.

Forty-nine patients with corpus callosum (CC) anomalies were evaluated in terms of the clinical features and magnetic resonance imaging (MRI) findings. CC anomalies were classified as CC agenesis: 6 (12%), CC hypogenesis: 5 (10%), and CC hypoplasia: 38 (78%). In the CC hypoplasia group the mean value of the genu thickness of the CC was 0.29 +/- 0.1 cm, which was less than the normal value of the age-matched normal children (normal range: 0.6-1.2 cm). The associated brain abnormalities were in five distinct groups: gray matter abnormalities, white matter abnormalities, midline brain structure defects, cortical atrophy, and encephalomalacia. There was no uniformity for the clinical spectrum of CC anomalies. Microcephaly, developmental delay and seizures were the prominent findings in patients. The clinical features were more severe in cases with associated brain anomalies.

Prophylactic therapy for hemophilia in a developing country, Turkey.

Prophylaxis has been practiced for many years in Europe and is gaining acceptance worldwide with current viral inactivation procedures. Unfortunately, the high cost of prophylaxis is currently the major obstacle to its implementation in developing countries such as Turkey. The aim of this controlled preliminary study is to evaluate the efficacy, safety, and feasibility of prophylaxis. Seven boys aged 1.5-7 years (5.0 +/- 1.8), who had severe hemophilia (six A, one B) received 20-50 IU/kg factor twice weekly and were followed up for 6-24 months (14.5 +/- 6.6). Intermediate concentrates have been used in hemophilia A and ultrapure product for hemophilia B. The data obtained for the same group of patients before prophylaxis were used as a control group. Another control group was selected in another group of 10 hemophiliacs, mean age 12.5, and received treatment on demand. During prophylactic treatment, the episodes of bleeding were decreased (from 10.5 +/- 3.2 to 4.5 +/- 3.6). Orthopedic and radiologic joint scores were stable (from 0 to 1 and from 1.1 +/- 1.2 to 1.0 +/- 1.5). The patients spent significantly fewer days in the hospital (from 18 +/- 12 to 0.7 +/- 0.6). None of the patients was infected with hepatitis A, hepatitis B, or human immunodeficiency virus. One patient was seroconverted with anti-hepatitis C virus in the third month of prophylaxis. Mean consumption of concentrates for prophylaxis was 3489 +/- 960 IU/kg per year compared with 2073 +/- 1302 in conventional therapy. Prophylaxis was superior to treatment on demand even when given in a twice-weekly period with intermediate concentrates. In Third World countries, prophylaxis should be tried at least in selected severely hemophilic children in order to prevent disabilities.

Large craniopharyngioma extending to the posterior cranial fossa.

Craniopharyngiomas are most commonly located extra-axially in the sellar or suprasellar area. They are benign but aggressive neoplasms. This paper reports an 8-year-old girl with a large craniopharyngioma originating from the suprasellar region and extending to the posterior cranial fossa down to the region of the foramen magnum.

Dysosteosclerosis: clinicoradiologic findings including brain MRI.

Dysosteosclerosis is a very rare bone dysplasia associated with sclerosis and platyspondyly. This paper reports the clinicoradiologic and MR imaging findings in this rare condition. The primary brain MR imaging finding was retarded white matter matter myelination.

Cerebral astroblastoma resembling an extra-axial neoplasm.

A case of a cerebral astroblastoma is described in which MR imaging findings suggested the diagnosis of an extra-axial neoplasm. The lesion was proven to be intracerebral both surgically and histopathologically. Calvarial erosion, and buckling of the cerebral cortex by a peripherally located well circumscribed, highly enhancing mass, were the main MR findings leading to an errogenous preoperative diagnosis of an extra-axial mass. An astroblastoma should be included to the differential diagnosis of a superficially located tumor presenting with the findings of an extra-axial mass, especially in a young patient.