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1/5. Melanotic neuroectodermal tumor of infancy discovered after head trauma.

    Melanotic neuroectodermal tumor of infancy (MNTI) is a rare neoplasm that generally arises in the maxilla during the first year of life. Involvement of bones of the cranial vault or brain is extremely rare. We describe a 7-month-old black female who presented after falling out of bed onto a concrete floor. Subsequently, she developed an anterior frontal mass that enlarged over several days. Radiographs of the skull at her local hospital showed a depressed right frontal skull fracture. However, computerized tomography of the head (reviewed at our institution) revealed a slightly hyperdense extra-axial mass which crossed the anterior frontal midline, widening the metopic suture and extending into the anterior subgaleal scalp. hyperostosis of the adjacent frontal calvarium was also present. A craniotomy revealed a dark, 1.5-cm calcified epidural lesion with some features of an unusual hematoma. Microscopic evaluation revealed a chronic hematoma and MNTI. The tumor recurred within a year. MNTI should be included in the differential diagnosis of epidural and skull lesions in infants.
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ranking = 1
keywords = brain
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2/5. Depressed skull fractures: a pattern of abusive head injury in three older children.

    OBJECTIVE: To describe a pattern of abusive head injury in a series of children older than 4 years of age. methods: A hospital chart review of abused children with skull fractures from 1999 to 2001 was carried out. The clinical features, social background, and subsequent outcome and management are described. RESULTS: An 11-year-old girl and a pair of brothers of ages 7 and 9 were identified. The girl was attacked with a hammer during sleep by her stepmother, who committed suicide shortly afterwards. After craniotomy and intensive care, the child survived her multiple depressed fractures, intracranial bleeding, and brain contusion. Two brothers from a second family were attacked from behind with a hammer by their biological father, who was subsequently found to have undiagnosed schizophrenia. A depressed occipital fracture, without intracranial injury, was found in each child. The elder brother also had metacarpal fractures. Both children recovered without surgical intervention. CONCLUSION: A pattern of abusive head injury was described in older children with depressed skull fractures from blunt injury. The abusing parents were seriously mentally disturbed, and the abusive acts closely resembled child homicide.
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ranking = 1
keywords = brain
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3/5. The use of recombinant activated factor VII in three patients with central nervous system hemorrhages associated with factor vii deficiency.

    BACKGROUND: Recombinant activated factor VII (rFVIIa) is being tested to improve hemostasis in a variety of bleeding disorders. Clinical indications and efficacy are still being evaluated for this product. CASE REPORT: Over a 17-month period, rFVIIa was used to treat central nervous system hemorrhage in three patients who were found to have isolated FVII deficiency (21%, 40%, 27%). Patient A fell 30 feet, Patient B suffered a motor vehicle accident, and Patient C had a spinal cord hematoma. None of the patients had a history of bleeding diathesis. All three patients received rFVIIa after failing initial treatment with fresh-frozen plasma. RESULTS: Patient A was treated with 11 doses (initial dose 95 microg/kg; subsequent doses 8-38 microg/kg) over 10 days; Patient B received 13 doses (45-60 microg/kg) over 13 days; and Patient C received 5 doses (12-24 microg/kg) over 4 days. The prothrombin time corrected from 16.2 /- 1.8 (mean /- SD) to 11.2 /- 1.6 seconds after infusion of rFVIIa, but returned to pretreatment level in 14 /- 4 hours. At the cessation of therapy, all patients showed neurologic improvement. No complications related to the infusion of rFVIIa occurred. CONCLUSION: The use of rFVIIa may be of value both for its general effect on hemostasis, and specifically in the setting where there is a documented reduction in FVII. Doses lower than those used in patients with FVIII inhibitors appear to be effective in the setting of central nervous system hemorrhage.
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ranking = 4.3066484184629
keywords = nervous system, central nervous system
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4/5. poisoning or primary nervous system disease?--difficulties of the differential diagnosis exemplified by four different clinical cases.

    Acute or chronic injury of the nervous system caused by xenobiotics can resemble primary disorders of the nervous system. In this study, four different cases that are characterized by unclear clinical presentation have been discussed; they required a detailed differential diagnostics using modern radiologic and electrophysiologic studies. Case 1. A young alcohol abuser was referred to the Acute Poisonings Unit at Wroclaw with a presumptive diagnosis of methanol poisoning. Neither methanol nor ethylene glycol were detected in patient's serum and urine. During hospitalization in our ward he lost vision completely, and neurologic examination was consistent with a transverse spinal cord injury. Traumatic spinal cord injury coexisting with methanol poisoning, or even Devic's syndrome were considered in differential diagnosis. The MRI did not reveal a spinal cord injury, and the EMG showed severe demyelinating-axonal polyneuropathy. Finally the patient was diagnosed with methanol poisoning complicated by both loss of vision and severe alcoholic polyneuropathy. Case 2. A 27-year-old man was found unconscious in a street. A head CT revealed numerous small intracerebral hemorrhages, and patient's urine contained high concentration of amphetamine. A presumptive diagnosis of amphetamine poisoning complicated by intracranial hemorrhage was proposed. The repeat head CT revealed traumatic injury of the skull in a form of depression. Based on this result, the patient was diagnosed with a posttraumatic intracranial hemorrhage. Case 3. A young man with history of schizophrenia was transferred to our ward from a psychiatric hospital with a presumptive diagnosis of neuroleptic malignant syndrome complicated by rhabdomyolysis. infection of the nervous system and focal lesions in the brain were ruled out with help of lumbar puncture and a brain MRI. After having obtained additional details of patient's history, it appeared that the patient had not been taking neuroleptics, and therefore it was assumed that patient's condition be connected with a catatonic type of schizophrenia exacerbation. Case 4. A 17-year-old woman, who returned from a disco club, presented with a bizarre behavior, she spoke incoherently, and she saw everything in bright and intense colors. Then she experienced a severe seizure attack with loss of consciousness and apnea. Toxicologic tests were negative. The patient was referred to neurology where she was finally diagnosed with epilepsy, and the attack was induced by strobe lights in a disco club.
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ranking = 4.897884666772
keywords = brain, nervous system
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5/5. Effects of recombinant activated factor VII in traumatic nonsurgical intracranial hemorrhage.

    OBJECTIVE: To determine whether treatment with recombinant activated factor VII (rFVIIa) will prevent progression of bleeding in nonsurgical hemorrhagic traumatic brain injury (TBI). methods: Chart review from the trauma registry of a level 1 trauma center between January 1, 2002 and December 31, 2004 identified 2 patients who received rFVIIa for progressive hemorrhagic TBI. These patients were given a single dose of rFVIIa (120 mcg/kg) after a repeat head computed tomography (CT) scan showed worsening of intracranial bleeding. Pre-rFVIIa and post-rFVIIa coagulation parameters and postintervention CT scans were performed. A matched convenience sample was drawn from the institution's trauma registry reflecting similar injury patterns. RESULTS: The 2 patients who received rFVIIa were ages 61 and 79 years; the patients in the matched convenience sample were 57 and 63 years. Both sets of patients comprised 1 man and 1 woman who had suffered blunt trauma, including hemorrhagic TBI, and were matched according to age, gender, and injury severity score (ISS). During their hospital course, repeat CT scans documented worsening of intracranial hemorrhage in both cohorts. In the rFVIIa patients, follow-up CT showed overall improvement of head injury compared with the convenience sample. The rFVIIa patients also saw an appreciable decrease in both prothrombin time (PT) and international normalized ratio (INR). CONCLUSIONS: In hemorrhagic TBI, rFVIIa has the potential to limit or even halt the progression of bleeding that would otherwise place growing pressure on the brain. A prospective, randomized multicenter trial is planned to elucidate this hypothesis.
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ranking = 2
keywords = brain
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