Cases reported "Hydrocephalus"

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1/213. neurofibrillary tangles in the dementia of "normal pressure" hydrocephalus.

    Routine neuropathological examination could not explain the dramatic improvement exhibited by one patient with "normal pressure" hydrocephalus after shunting. The improved patient contrasted remarkably with the unchanged condition of four others also shunted successfully. The five brains were analysed by quantitative morphometry to determine the degree of neurofibrillary tangle formation in mesial temporal neurons. The density of tangle-bearing nerve cells in the four unimproved cases was markedly greater than in age-matched control brains from nineteen normal subjects, and fell in the same range as that of eight dements with neuropathologically confirmed Alzheimer's disease. The density of the one who recovered was within normal limits. The duration of dementia before shunting, and the total duration of dementia in these five patients rank in the same order as their degree of neurofibrillary formation. Furthermore, a positive linear correlation exists between the Tangle Indices and the total duration of dementia. The data suggest that early diagnosis may improve the chances of reversing the dementia of normal pressure hydrocephalus before histological alterations prove too severe.
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2/213. A case of cerebrospinal fluid eosinophilia associated with shunt malfunction.

    A 3-month-old female patient presented with a meningomyelocele at the lumber region associated with congenital hydrocephalus. She underwent ventriculoperitoneal (V-P) shunt surgery using the Sophy system. The shunt system was replaced due to a malformation. Following replacement, the patient presented with cerebrospinal fluid (CSF) eosinophilia at the age of 8 months. The eosinophilic granulocytosis of the CSF improved dramatically following systemic prednisolone administration. CSF eosinophilia without accompanying inflammation or pyrexia in the present case may have resulted from an allergic response to a foreign material such as the silicone tube pressure valve of the Sophy system or the sutures rather than bacterial or fungal infection. Based on our results, we believe that some patients may experience CSF eosinophilia following postoperative V-P shunt due to an allergic reaction to the shunt equipment. Prompt steroid treatment can produce spontaneous regression in such cases.
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3/213. Treatment of a symptomatic posterior fossa subdural effusion in a child.

    We describe the first observation of a child with a posterior fossa subdural effusion with secondary hydrocephalus and tonsillar herniation. We diagnosed this entity in a 14-month-old girl with no history of trauma or coagulation disorder. The patient presented in our emergency department with opisthotonus and raised intracranial pressure resulting from supratentorial hydrocephalus. An emergency ventriculo-peritoneal shunt was placed, which resolved the symptoms only temporarily. Eventually external drainage of the subdural fluid was performed. The collection gradually disappeared, and both the external subdural shunt and the ventriculo-peritoneal shunt were removed. The patient made a complete neurological recovery. We review the physiopathology and treatment of subdural effusions in general, and propose some guidelines for the management of symptomatic effusions occurring in the posterior fossa in particular.
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4/213. spinal cord hamartoma: case report.

    OBJECTIVE AND IMPORTANCE: spinal cord hamartomas are infrequently mentioned in the literature. The authors present a unique report detailing the clinical presentation of a spinal cord hamartoma, with supporting radiographic and pathological data. CLINICAL PRESENTATION: A 26-year-old man presented with progressive right upper extremity weakness. Imaging studies revealed an exophytic cervical spinal cord mass. INTERVENTION: Open biopsy was undertaken and revealed tethering of the lesion to the dura. A pathological examination revealed a spinal cord hamartoma. CONCLUSION: The patient's symptoms improved postoperatively, suggesting that tethering of the spinal cord was responsible for the symptoms. Although unusual, hamartoma should be included in the differential diagnosis of an exophytic spinal cord lesion.
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5/213. prenatal diagnosis in a family with severe type I plasminogen deficiency, ligneous conjunctivitis and congenital hydrocephalus.

    Severe type I plasminogen deficiency may cause severe ligneous conjunctivitis, a rare and unusual form of chronic pseudo-membranous conjunctivitis that usually starts in early infancy, but also pseudo-membranous lesions of other mucous membranes in the mouth, nasopharynx, trachea and female genital tract, and in rare cases congenital occlusive hydrocephalus. The index patient, the daughter of a consanguineous marriage, had suffered from severe ligneous conjunctivitis and had died from decompensated congenital hydrocephalus despite numerous shunt revisions. She was found to be homozygous for a non-sense mutation in exon 15 of the plasminogen gene (Trp597->Stop). In her next pregnancy, the mother asked for prenatal diagnosis of the plasminogen deficiency. Chorionic villus biopsy was performed at 12 weeks of gestation. dna analysis of the plasminogen gene by PCR and single-strand conformation polymorphism (SSCP) revealed that the fetus exhibited an identical heterozygous band pattern as observed in the healthy mother. Therefore, the fetus was heterozygous for the Trp597->Stop mutation in plasminogen exon 15. In addition, the fetus was found to be male by cytogenetic analysis and by multiplex PCR analysis using two polymorphic X-chromosomal markers (DXS424, HPRT). These findings excluded the possibility of contamination by maternal dna. It was concluded that the fetus was not at risk for ligneous conjunctivitis and its associated complications. After the birth of a healthy boy, plasminogen functional activity was shown to be 38 per cent. dna analysis confirmed prenatal molecular genetic results.
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6/213. Chronic hydrocephalus and suprasellar arachnoid cyst presenting with rhinorrhea.

    Spontaneous CSF leak with rhinorrhea may be secondary to many intracranial congenital and acquired conditions. However, no cases of chronic hydrocephalus and suprasellar arachnoid cyst presenting with rhinorrhea as the unique clinical manifestation are reported in the literature. A 29-year-old-man with four-month history of episodic rhinorrhea had a large suprasellar arachnoid cyst with chronic hydrocephalus on magnetic resonance. Endoscopic ventricular fenestration of the cyst failed to obtain remission of the CSF leak, because it was not possible to fenestrate the cyst with the almost completely obliterated suprasellar cistern. Clinical remission occurred after restoration of the CSF flow from the cyst to the cisternal spaces by a direct approach. The CSF leak in this case was secondary to the chronic compression over the dural and bone structures of the sellar region by the cyst or chronic hydrocephalus.
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7/213. Subdural empyema complicating cerebrospinal fluid shunt infection.

    Subdural empyema has not been reported previously as a complication of cerebrospinal fluid (CSF) shunt surgery. An infant submitted to CSF shunt insertion for congenital hydrocephalus developed subdural empyema after a failed attempt to treat a superficial scalp wound infection with oral antibiotics. enterobacter cloacae was isolated from the empyema. Temporizing management of the preceding superficial wound infection with oral antibiotics probably was the cause of this exotic pathogen. The treatment of infected scalp wounds contiguous with shunt hardware must be surgical.
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8/213. Pressure in the hydrocephalic fetal head during the first stage of labor.

    Intraamniotic pressure was studied in the 30th week of amenorrhea in relationship with fetal intracranial pressure with open-tip catheters. The fetus had a severe hydrocephalus (echoscopy 16 cm) due to a teratologic malformation of the cerebrum. Clinically nonoperative treatment was indicated. intracranial pressure (X) was invariably higher than intraamniotic pressure (Y) between contractions: Y = 2.04 0.54 X, and during contractions: Y = 5.30 0.55 X. There was no definite relationship between intrauterine and intracranial pressure, and the fetal tachogram. A definite relationship was established with the supine position of the patient and decelerations in the fetal tachogram. It is suggested that when fetal cardiac decelerations are seen during the first stage of labor it seems advisable to look for factors such as umbilical cord compression and decrease of materno-placental perfusion rather than fetal head compression.
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9/213. Spontaneous epidural hematoma following a shunt in an infant with congenital factor x deficiency. Case report and literature review.

    The authors describe a case of an infant with congenital factor x deficiency. The patient presented with a central nervous system hemorrhage followed by hydrocephalus. He underwent a ventriculoperitoneal shunt and, during the postoperative period, developed a spontaneous epidural hematoma, which was evacuated. The clinical and pathophysiological aspects of this case are discussed based on a literature review.
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10/213. Symptomatic calcified subdural hematomas.

    Two unique cases of chronic calcified subdural hematomas are reported in children as a long-term complication of a ventriculoperitoneal shunt. Both the patients had undergone shunt procedures in infancy for congenital hydrocephalus. In one patient, the cause of the hydrocephalus was aqueduct stenosis, while in the second patient, a lumbar meningomyelocele was associated with hydrocephalus. In both these patients, a ventriculoperitoneal shunt was done in infancy. In one of them, following the shunt surgery, a bilateral subdural collection was noticed which required burr hole evacuation. Both the patients remained asymptomatic for 9 years, when they presented to our center with acute raised intracranial pressure and contralateral hemiparesis. Both the patients had a relatively short history and had altered sensorium at admission. Surprisingly, in both the patients, the CT scan showed significant mass effect producing calcified subdural hematomas. The shunt systems were found to be working well at surgery. craniotomy and excision of the calcified subdural hematomas was undertaken. Postoperatively, the patients showed satisfactory recovery, and at discharge the patients were doing well. At the follow-up at the outpatient clinic, the patients were asymptomatic.
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