11/134. Neuropsychiatric manifestations of a third ventricular colloid cyst. A 48-year-old man with no psychiatric history presented with memory disturbances, personality changes, emotional lability and mild paranoia with no evident mood disturbances. A colloid cyst of the third ventricle was discovered and surgically excised. ( info) |
12/134. Isolated large third-trimester intracranial cyst on fetal ultrasound: fact or fiction? OBJECTIVE: To distinguish the fact from artifact of an isolated, large, intracranial cyst on prenatal sonography (PSG). BACKGROUND: The use of PSG is rapidly increasing with most obstetric ultrasounds occurring in general community settings like small hospitals and clinics with personnel who have variable training, experience, and interest levels. In contrast, most PSG articles and books are produced in large subspecialty centers with concentrated referral bases plus both highly-trained and experienced personnel. DESIGN/methods: We report a series of 2 normal newborn patients who had a large prenatal unilateral intracranial cyst diagnosed by PSG in the 10 years between July of 1989 and 1999 at a rural community hospital. The newborns had imaging studies at birth and their neurodevelopmental progress was followed for several years. Textbook, bibliography and computerized medline (1966-present) searches including prenatal ultrasound, observer variation, diagnostic errors, reproducibility of results, sensitivity and specificity, accuracy, central nervous system, false-positive, prenatal diagnosis, and brain were examined starting in August 1996 for reports. RESULTS: There were 4079 obstetric ultrasounds performed in 3.5 years, January 1996 through July 1999 at this rural community facility. This rate extrapolates to a total of 11 654 obstetric ultrasounds over the 10-year study period in which the 2 cases of intracranial cyst artifact occurred. Thus, the incidence of 2 intracranial cyst artifacts was estimated as 2/11 654 PSG, a .0002% false-positive rate. CONCLUSIONS: This is the first report of the occurrence of PSG artifacts in a community facility. Artifact is a real problem and needs to be specified in differential diagnoses. There are ways to decrease sonographic artifact-or at least to recognize it-so our estimates at a community hospital for its occurrence are presented with the relevant technical and ethical issues. None of these issues have been previously reported in the pediatric literature. Our false-positive rate for large intracranial cyst compares favorably with other reports. Our estimate may inflate our denominator by reporting scans rather than the number of fetuses scanned, and our numerator may miss cases that moved from the community. confusion differentiating PSG artifact from reality often occurs when interpreting static or frozen real-time images. The signs that sonogram images may be artifacts include defects that: extend outside the fetal body; change shape, size and echogenecity with different scan planes; are not seen on all examinations; and are isolated in an otherwise normal fetus. Failure to offer quality PSG in clinical settings where it is available restricts access of pregnant women to the diagnosis of fetal anomalies, and therefore restricts access to the options of pregnancy termination, fetal therapy like fetal surgery, and delivery options of timing, setting, and mode. We suggest a multidisciplinary approach to prenatal abnormalities like isolated third trimester unilateral intracranial cyst in both primary and tertiary care settings aids interpretation followed by expectant conservative management without elaborate, risky, or terminal interventions. ( info) |
13/134. Symptomatic ependymal cysts of the perimesencephalic and cerebello-pontine angle cisterns. Primary intracranial ependymal cysts are extremely rare. These are congenital, benign ependyma lined, commonly intraparenchymal and uncommonly extraparenchymal cysts in leptomeningeal location of variable size seen in the adult life.The authors report two cases of symptomatic ependymal cysts: one in the perimesencephalic cistern in a 10-month-old infant presenting with delayed mile stones and another in the cerebello-pontine cistern in a morbidly obese 35-year-old woman with known benign intracranial hypertension now presenting with hemifacial spasm. Neurosurgical intervention was curative in both cases. Interesting clinical, radiological and histological features are presented and discussed. ( info) |
14/134. Merosin-deficient congenital muscular dystrophy with mental retardation and cerebellar cysts unlinked to the LAMA2, FCMD and MEB loci. We report a case of congenital muscular dystrophy with secondary merosin deficiency, structural involvement of the central nervous system and mental retardation in an 8-year-old girl from a consanguineous family. She had early-onset hypotonia, generalized muscle wasting, with weakness especially of the neck muscles, joint contractures, mental retardation and high creatine kinase. Muscle biopsy showed dystrophic changes with partial deficiency of the laminin alpha(2) chain. Cranial magnetic resonance imaging revealed multiple small cysts in the cerebellum, without cerebral cortical dysplasia or white matter changes. The laminin alpha(2) chain (6q2), Fukuyama type congenital muscular dystrophy (9q31-q33) and muscle-eye-brain disease (1p32-p34) loci were all excluded by linkage analysis. We suggest that this case represents a new entity in the nosology of congenital muscular dystrophy. ( info) |
15/134. syndrome of inappropriate secretion of antidiuretic hormone (SIADH) and adrenal insufficiency induced by rathke's cleft cyst: a case report. We report a case of a seventy-year-old woman with syndrome of inappropriate secretion of antidiuretic hormone (SIADH) and adrenal insufficiency induced by Rathke's cleft cyst. She experienced nausea, vomiting, diarrhea, and headache and disturbance of consciousness induced by hyponatremia at a serum sodium level of 100 mEq/l. In spite of severe hyponatremia, urinary sodium excretion was not suppressed and serum osmolality (270 mOsm/kg) was lower than urine osmolality (304 mOsm/kg), and arginine vasopressin (AVP) remained within normal range. SIADH was diagnosed because she was free from other diseases known to cause hyponatremia such as dehydration, cardiac dysfunction, liver dysfunction, renal dysfunction, hypothyroidism, and adrenal insufficiency. Cranial computed tomographic (CT) scan and cranial magnetic resonance (MR) imaging showed a cystic lesion of approximately 2 cm in diameter in the pituitary gland. These images suggested that the cystic lesion was a Rathke's cleft cyst, which was the cause of SIADH. water restriction therapy normalized her serum sodium concentration and improved her symptoms. After one year, she suffered from general fatigue, appetite loss, fever, and body weight loss (5 kg/2 months). She had neither hypotension nor hypoglycemia, but her serum sodium level was low and serum cortisol, ACTH, and urine free cortisol were very low. Therefore, secondary adrenal insufficiency was suspected and diagnosed by stimulation tests. After start of hydrocortisone replacement therapy (10 mg/day), her symptoms disappeared. In conclusion, Rathke's cleft cyst should be kept in mind as a potential cause in a patient with SIADH, hypopituitarism, and/or adrenal insufficiency. ( info) |
16/134. Rathke's cleft cyst abscess. Pituitary abscesses are rare. Occasionally they will arise in pre-existing pituitary pathology. We report such an occurrence within a Rathke's cleft cyst. On the basis of history and imaging, this was indistinguishable from more commonly encountered pituitary pathology. ( info) |
17/134. Traumatic porencephalic cyst and cholesteatoma of the ear. Porencephalic cyst expanding into the ear is a very rare complication of temporal bone fracture. We report a case of a 20-year-old male who developed a traumatic porencephalic cyst of the temporal lobe expanding into the ear through a tegmen fracture in association with a cholesteatoma. The clinical presentation was otitic meningitis. This occurrence was not encountered in any of the cases reviewed in the literature. The diagnosis, pathogenesis, treatment and prognosis of this condition are reviewed. ( info) |
18/134. Split cord malformation and neurenteric cyst--case report and a theory of embryogenesis. A rare case of split cord malformation with neurenteric cyst is presented. The clinical, radiological and surgical findings are presented, and the possible embryological basis is discussed. To our knowledge, only seven such cases have been reported in the literature. ( info) |
19/134. Rathke's cleft cyst presenting as sphenoid sinusitis. Rathke's cleft cysts are developmental abnormalities of the craniopharyngeal duct composed of retained stratified ciliated cuboidal cells of the respiratory type. Incidentally found in the pars intermedia in 13% to 33% of routine autopsies, they are discovered in growing numbers on CT and MRI scans. From 1991 to 1999, 9 cases of Rathke's cleft cysts were encountered by the senior authors, 4 of which were treated surgically (3 by using a transsphenoidal approach and one by using a transcranial subfrontal approach). Of the 3 patients treated with a transsphenoidal approach, 2 presented with symptoms mimicking sphenoid sinusitis and were initially referred for otolaryngologic evaluation. The clinical, pathologic, and radiologic features, as well as management and follow-up of those 2 patients, are reviewed. There has been only one previous case report of Rathke's cleft cysts presenting as sinusitis. Nevertheless, the otolaryngologist should be aware of this condition because it may present with other significant symptomatic extracranial extensions. ( info) |
20/134. galactorrhea as the sole presenting symptom of a posterior third ventricular epidermoid cyst. BACKGROUND: brain tumors that are remote from the sellar and parasellar region rarely produce galactorrhea. CASE DESCRIPTION: galactorrhea was the sole presenting symptom in a patient with a posterior third ventricular epidermoid cyst. On investigation, her serum prolactin level was mildly elevated. After radical excision of the tumour, the spontaneous galactorrhea stopped and the serum prolactin level decreased. CONCLUSION: Because of the clinical and biochemical response seen after surgery, we speculate that the galactorrhea was caused by the presence of the tumor. We postulate that the pressure exerted by the tumor on the diencephalic structures was probably the cause of galactorrhea in our patient. ( info) |