Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0030552 (paresis)
 5,831 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Five children, three girls and two boys, were treated for precocious puberty secondary to hypothalamic hamartoma by resection of the hamartoma. The patients' ages at onset of pubertal development ranged from 6 to 19 months. The hamartomas ranged in size from 6 to 10 mm; four were pedunculated, one was sessile, and all were located below the tuber cinereum. The hamartomas were excised via a right subtemporal approach, with transection at the inferior surface of the hypothalamus; two were adherent posteriorly to the basilar artery and brain stem, and the adhesions were divided. Postoperatively, three children exhibited a transient oculomotor paresis and one other child required eye-muscle surgery. The symptoms and signs of precocious puberty completely regressed postoperatively in all patients. Preoperative hormone assays of testosterone, luteinizing hormone, and follicle-stimulating hormone were within the pubertal range in all five children; postoperative assays fell to prepubertal levels. The children have been followed for 0.5 to 10.5 years (mean 5.0 years) postoperatively, without evidence of recurrence of precocious puberty. One child has begun spontaneous puberty at a normal age. It is concluded that complete resection of hypothalamic hamartomas causing precocious puberty is curative.
 ...
PMID:Neurosurgical treatment of hypothalamic hamartomas causing precocious puberty. 841 46

A series of 17 patients aged from 9 months to 32 years with refractory epilepsy due to hypothalamic hamartoma were treated by total removal (one case) and disconnection (16 cases) between 1997 and 2002. The mean age at seizure onset was 16 months. Sixteen patients had gelastic seizures, 14 had partial seizures and three had generalized tonic-clonic seizures. The mean seizure frequency was 21 per day. Four patients had borderline intelligence quotient and the others were mentally retarded. Five patients presented with precocious puberty, one with acromegaly, and four suffered from obesity. Brain magnetic resonance imaging, performed at least twice in each patient, showed the hamartoma as a stable homogeneous interpeduncular mass implanted either on the mammilary tubercle or on the wall of the third ventricle with variable extension to the bottom. Ictal single photon emission computed tomography, performed in four patients, showed hyperperfusion within the hamartoma in two patients. Twenty-five operations were performed in the 17 patients. The first patient underwent total removal of the hamartoma, whereas the following 16 patients underwent disconnection through open surgery (14 procedures) and/or endoscopy (9 procedures). Eight patients became seizure-free, one patient had only brief gelastic seizures, and eight patients were dramatically improved with a mean follow up of 18.6 months (8 days to 43 months). Surgery was safe in all but two patients: the first patient had transient hemiplegia and the third cranial nerve paresis, and the other developed hemiplegia due to ischemia of the middle cerebral artery territory. The quality of life, and behavior and school performance were greatly improved in most patients. Our series illustrates the feasibility and relative safety of disconnection surgery for hypothalamic hamartomas with seizure relief in 53% of patients and dramatic improvement in the others. Surgical observations led us to propose a new anatomical classification according to the anatomical relationship between the hamartoma and the adjacent hypothalamus and third ventricle. Endoscopic disconnection seems to be a very safe way to treat hamartomas in intraventricular locations.
 ...
PMID:Disconnecting surgical treatment of hypothalamic hamartoma in children and adults with refractory epilepsy and proposal of a new classification. 1262 81

The authors present three boys--3 years old, 5.8 years old and 10.4 years old--who were diagnosed with isosexual precocious puberty (IPP) triggered by a rare developmental disorder of suprasellar arachnoid cyst (SAC) accompanied by corpus callosum and fornix dysgenesis as well as anterior commissura magna agenesis (patient 1) and empty sella (patients 2, 3). The reason for diagnostic management recommendation was a rapid progression of IPP signs over one year (patients 1, 2) or 6 months (patient 3) prior to hospitalization, these signs having been present but less intense since infancy (patient 1), 4th year of life (patient 2) and approximately 8 years of age (patient 3). Neurological signs (spastic paresis in patient 1, postural tremor in patient 2 and head bobbing and behavioral changes in patient 3), as well as slowly progressing increased head circumference were observed since neonatal period (patient 1), 1 year old (patient 2) and approximately 4 years old (patient 3). None of the patients manifested hypophyseal-hypothalamic axis dysfunction other than IPP prior to and after surgical management. Shunt implantation resulted in gradual resolution of neurological signs in all patients and in patient 3 also in partial normalization of serum testosterone levels and growth rate. Regression of IPP in patients 1 and 2 was achieved by administration of a long-acting GnRH analogue. Our observations are in accord with data reported by other investigators and confirm the often slow, insidious development of subsequent SAC signs, the type and intensity of which differ from patient to patient. We suggest that some of the neuroanatomical anomalies coexisting with SAC may have a common genesis, or they could under certain conditions be an additional trigger for IPP and possibly other hypothalamopituitary dysfunction.
 ...
PMID:Suprasellar arachnoidal cyst as a cause of precocious puberty--report of three patients and literature overview. 1270 72