1/21. Bilateral sensorineural deafness, partial agenesis of the corpus callosum, and arachnoid cysts in two sisters.We describe two sisters (ages 10 and 3 years, respectively) with a normal development and a combination of congenital sensorineural hearing loss, partial agenesis of the corpus callosum, arachnoid cyst, and hydrocephalus. Neither girl has distinctive physical anomalies. In the oldest girl, there was a hearing loss of 80 dB bilaterally, and the most severe loss on audiogram was seen at 2,000-4,000 Hz. In the youngest girl, there was a hearing loss of 100 dB bilaterally. Above 2,000 Hz no neural reactions were seen. Cerebral magnetic resonance imaging in one girl and computed tomography in the other showed a partial agenesis of the corpus callosum and a cyst in the pineal region, causing an aqueduct stenosis by compression and consequent hydrocephalus. The parents have normal hearing, and brain magnetic resonance imaging showed no abnormalities. They are nonconsanguineous but from the same small village. This is the first report of a combination of congenital sensorineural hearing loss, partial agenesis of the corpus callosum, and an arachnoid cyst. The pattern of inheritance is probably autosomal recessive.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
2/21. Acute ventricular hemorrhage in adults with hydrocephalus managed by corpus callosotomy and fenestration of the septum pellucidum. Report of three cases.Three patients with hypertension-induced basal ganglia or thalamic hemorrhage and ventricular rupture underwent corpus callosotomy and fenestration of the septum pellucidum. A patient with a left thalamic hemorrhage underwent surgery on an emergency basis and made a complete physical recovery, although she retained mild psychomotor deficits. Another patient with a large right basal ganglia hemorrhage who also underwent surgery on an emergency basis retained a spastic left hemiparesis without evident psychomotor deficits. The third patient with a left thalamic and basal ganglia hemorrhage, who was initially awake and then lapsed into stupor days later, underwent surgery, but did not recover consciousness. hydrocephalus was reversed and effectively controlled in all three patients without having to perform a shunt placement procedure.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
3/21. Hunter syndrome presenting as macrocephaly and hydrocephalus.A 2-year-old boy with macrocephaly, communicating hydrocephalus, and mild hepatosplenomegaly was found to have mild Hunter syndrome (MPS II). Establishment of the latter diagnosis was complicated by the paucity of obvious physical findings because of the patient's young age and his ethnic origin.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
4/21. Brain tumor presenting as anorexia nervosa in a 19-year-old man.Slow-growing brain tumors can produce disturbances of food intake and endocrine dysfunction. We report a case of slow-growing midline brain tumor in a patient with clinical presentation of anorexia nervosa (AN). A 19-year-old man was referred from a general practitioner to a psychiatric clinic due to illness behavior and psychopathological characteristics of AN. His body weight had decreased from 52 kg to 40 kg within 6 months. Laboratory tests showed hypernatremia (160 mmol/L), adrenal insufficiency (adrenocorticotrophic hormone, 11.4 pg/mL; 8 am cortisol, 1.4 microg/dL; 4 pm cortisol, 11.4 microg/dL) and hypogonadotropic hypogonadism (testosterone < 0.5 ng/mL, follicle-stimulating hormone < 0.1 mIU/mL, luteinizing hormone < 0.7 mIU/mL). Brain magnetic resonance imaging showed an extensive mass lesion at suprasellar, hypothalamic region, third ventricle, pineal region, lateral ventricle, and corpus callosum. Owing to central herniation during physical assessment, he died of unknown intracranial pathology. This case suggests that an intracranial tumor near the hypothalamus should be included in the differential diagnosis of AN. Any male adolescent with the clinical impression of AN should receive periodic re-evaluation, including neurological, endocrinological and, if necessary, neuroimaging study.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
5/21. laparoscopy in the diagnosis and treatment of malfunctioning ventriculo-peritoneal shunts in children.Abdominal complications following ventriculo-peritoneal shunting for hydrocephalus are not uncommon. One of the complications that has heretofore required abandonment of the peritoneal shunt has been entrapment or encystation of the peritoneal limb of the catheter system. Four infants have been identified with malfunction of the peritoneal limb of the system by characteristic physical findings of increasing head size and dissection of fluid along the shunt track. Abdominal radiographs in multiple views revealed the tip of the peritoneal catheter to be fixed in position in each patient and sonography aided in identification of encysted catheters. These infants underwent six laparoscopic procedures to define the cause of shunt malfunction and to reposition the catheter. cerebrospinal fluid cysts were encountered in three infants and entrapment by the falciform ligament in the fourth. All catheters were easily repositioned within the abdominal cavity with laparoscopy forceps and three of the infants presently have normally functioning shunt systems.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
6/21. Premeditated assaults on young boys by a man with spina bifida and hydrocephalus--a cognitive-behavioural approach to treatment.This paper describes the case of a 25-year-old man with spina bifida and hydrocephalus, who had at least a ten-year history of physically assaulting young boys. Anti-social behaviour is reported as comparatively rare in people with this disability, and the case study illustrates how psychological assessment and treatment techniques were successfully applied. Information from his personal history plus a functional analysis of the assaults suggested that a combination of existing psychological difficulties and learning experiences contributed to the development of his behaviour. A behavioural model of the psychological mechanisms underlying the behaviour was suggested and treatment objectives defined. Cognitive and behavioural treatment techniques were adapted where necessary, with a good outcome. A gap in services for physically handicapped people with behavioural problems is noted and briefly discussed.- - - - - - - - - - ranking = 2keywords = physical (Clic here for more details about this article) |
7/21. Long-term survival following treatment of multiple supra- and infratentorial aspergillus brain abscesses.aspergillus brain abscess is a rare but frequently fatal disease. Despite the scarcity of reported survivors, a combination of medication and surgical treatment might be effective. We report a 37-year-old man who developed multiple aspergillus brain abscesses after severe bacterial pneumonia. The following strategy was used to treat the patient: diagnostic puncture of one of the abscesses, long-term treatment with medication, excision of chronic granuloma in the occipital lobe and fourth ventricle, surgical treatment of the hydrocephalus. Following various surgical and antifungal treatments, the patient survived. Nearly three years after discharge, he still is in good physical condition and has a moderate neurologic deficit. Only 36 patients have been reported to have survived longer than three months after receiving treatment for brain aspergillosis. A course of medication in combination with various surgical procedures was required to achieve a successful outcome in this otherwise fatal disease.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
8/21. Meckel-gruber syndrome associated with gastrointestinal tractus anomaly.Meckel-Gruber syndrome (MGS) is rare autosomal recessive disorder characterized by occipital encephalocele, postaxial polydactyly and polycystic kidneys. A one-day-old girl was admitted to our clinic with occipital encephalocele, polydactyly, ulnar deviation of left hand and failure to thrive. Patient's parents were first-degree relatives. It was learned that the patient's two sisters had died from similar anomalies. In our case, prenatal sonographic examination revealed oligohydramnios and hydrocephaly in the 33rd week of gestation. At birth her weight was 2200 g. Both physical and radiological examinations diagnosed MGS. Cranial computed tomography (CT) showed agenesis of cerebellar vermis and corpus callosum, and cystic dilatation of the 4th ventricle and lateral ventricles. The case died due to severe respiratory distress in the intensive care Unit on day 38. In the postmortem examination, longitudinally located intestine-like stomach was determined without a fundus. In conclusion, intestinal malrotation and hepatic portal fibrosis have been reported in MGS in the literature. In this case, a longitudinally located intestine-like stomach in MGS is reported for the first time. No such association to our knowledge has been previously reported.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
9/21. Pericallosal artery pseudoaneurysm secondary to endoscopic-assisted ventriculoperitoneal shunt placement. Case report.Complications following cerebrospinal fluid (CSF) diversion procedures are protean. The formation of pseudoaneurysms after the placement of a ventricular catheter as part of a CSF diversion procedure is presumably quite rare. The authors report the case of a child in whom a pericallosal artery pseudoaneurysm developed following the endoscopic insertion of a ventricular catheter as part of a ventriculoperitoneal shunt placement procedure. Significant intraventricular bleeding signaled vascular injury. angiography revealed a right pericallosal artery pseudoaneurysm. The patient subsequently underwent surgical trapping of his pseudoaneurysm and physical therapy for left leg monoparesis. This appears to be the first reported case of pseudoaneurysm formation following the placement of a ventricular catheter for a CSF diversion procedure. This case underlines a rare but serious risk involved with the routine placement of CSF shunts.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
10/21. Spontaneous bacterial peritonitis causing serratia marcescens and proteus mirabilis ventriculoperitoneal shunt infection. Case report.The authors report their experience treating a polymicrobial ventriculoperitoneal (VP) shunt infection in a developmentally delayed 21-year-old woman. cerebrospinal fluid (CSF) cultures grew serratia marcescens and proteus mirabilis. On admission and throughout her hospitalization, results of physical examination of her abdomen were normal, and radiographic studies showed no evidence of bowel perforation or pseudocyst formation. Contrast-enhanced computed tomography of the abdomen revealed a small fluid collection. After a course of intravenous gentamicin and imipenem with cilastatin in conjunction with intrathecal gentamicin, the infection was resolved and the VP shunt was reimplanted. Although VP shunt infections are not uncommon, S. marcescens as a causative agent is exceedingly rare and potentially devastating. Only two previous cases of S. marcescens shunt infection have been reported in the literature. Authors reporting on S. marcescens infections in the central nervous system (CNS) have observed significant morbidity and death. Although more common, the presence of P. mirabilis in the CSF is still rare and highly suggestive of bowel perforation, which was absent in this patient. Spontaneous bacterial peritonitis was the likely source from which these bacteria gained entrance into the VP shunt system, eventually causing ventriculitis in this patient. The authors conclude that in light of the high morbidity associated with S. marcescens infection of the CNS, intrathecal administration of gentamicin should be strongly considered as part of first-line therapy for S. marcescens infections in VP shunts.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
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