Cases reported "Nervous System Diseases"

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1/42. Treatment of delayed-onset neurological deficit after aortic surgery with lumbar cerebrospinal fluid drainage.

    OBJECTIVE: The phenomenon of delayed neurological deficit after thoracoabdominal aortic aneurysm repair was first reported in the late 1980s. The mechanism may be reduced collateral circulation during periods of hypotension, cord edema, or reperfusion injury. Few patients with delayed-onset neurological deficit have recovered from this devastating complication. The experience with six patients treated with lumbar cerebrospinal fluid (CSF) drainage is reported. methods: Five patients underwent thoracoabdominal aortic aneurysm repair. Before and immediately after the operation, the patients exhibited no abnormalities in motor or sensory function. patients presented between 12 and 40 hours postoperatively with rapid motor and sensory loss in their lower extremities. Hypotensive events immediately preceded the onset of deficit in five patients. The sixth patient experienced an acute onset of back pain and was found to have thrombus without evidence of dissection in the descending aorta. RESULTS: patients were treated with volume expansion and vasoactive drugs to achieve a mean arterial pressure of more than 70 mm Hg. Lumbar CSF drainage was instituted promptly in four patients; all displayed marked neurological improvement. Two patients underwent CSF drainage several hours after the onset of symptoms and did not improve. The duration of CSF drainage ranged from 15 to 72 hours, with a goal of maintaining the lumbar CSF pressure at less than 10 mm Hg. CONCLUSION: The efficacy of CSF drainage may relate to reducing CSF pressure, which may increase spinal cord perfusion. Rapid initiation of CSF drainage with aggressive support of blood pressure may result in neurological improvement in some patients.
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2/42. Ophthalmologic involvement in the syndrome of headache, neurologic deficits, and cerebrospinal fluid lymphocytosis.

    PURPOSE: To emphasize that papilledema and other ophthalmic manifestations may occur in the syndrome of headache, neurologic deficits, and cerebrospinal fluid lymphocytosis (HaNDL). DESIGN: Two interventional case reports. methods: Two patients were seen with ophthalmologic findings, including decreased vision, papilledema, sixth nerve palsy, and a variety of neurologic deficits. Each underwent cerebrospinal fluid analysis and intracranial pressure measurement by spinal tap and neuroimaging studies to confirm the diagnosis of HaNDL. RESULTS: Both patients received acetazolamide to lower intracranial pressure. The first patient had complete resolution of signs and symptoms. The second, who was also given systemic corticosteroids, was left with diminished visual acuity in the right eye with nasal visual field loss and optic atrophy. CONCLUSIONS: The diagnosis of HaNDL is one of exclusion, which must be made in conjunction with a neurologist. HaNDL may be accompanied by elevated intracranial pressure and papilledema. As in other disorders causing papilledema, these patients may have permanent visual sequelae. Recognition by the ophthalmologist of this rarely reported syndrome will facilitate prompt patient diagnosis and treatment.
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3/42. Transient neurologic deficits associated with carbamazepine-induced hypertension.

    carbamazepine is a well-established, effective treatment of complex partial seizures and is well tolerated in most patients. The adverse effects of carbamazepine include aplastic anemia, agranulocytosis, pancytopenia, bone marrow depression, thrombocytopenia, cardiac conduction abnormalities, congestive heart failure, and peripheral edema. hypertension or hypotension has also rarely been documented in patients with either therapeutic or toxic blood levels of carbamazepine. It is possible that carbamazepine-induced hypertension in those with therapeutic blood levels is rarely seen because most of the patients who begin treatment are young and do not have baseline hypertension. The authors describe a patient of African-American descent with a history of controlled essential hypertension who developed severe uncontrolled hypertension when started on carbamazepine. Treatment with additional antihypertensive medications did not reduce his blood pressure. In addition, he developed two episodes of transient neurologic deficits, the symptoms of which consisted of dysarthria, vertigo, and unstable gait. A substantial reduction of his carbamazepine dose resulted in the control of his blood pressure and no recurrence of his symptoms.
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4/42. Posterior reversible encephalopathy syndrome (PRES) in critically ill obstetric patients.

    OBJECTIVE: To describe clinical, neuroradiological and evolutionary findings in obstetric patients with posterior reversible encephalopathy syndrome (PRES). DESIGN: Retrospective case series. SETTING: University intensive care unit (ICU). patients: Four critically ill patients. Two patients experienced PRES in late postpartum without the classical pre-eclamptic signs. All patients showed impairment of consciousness and epileptic seizures; two of them presented cortical blindness and headache, too. True status epilepticus (SE) occurred in two cases. In all patients MRI showed the typical feature of gray-white matter edema, mainly localized to the temporo-parieto-occipital areas. INTERVENTIONS: Normalization of high blood pressure (BP) and treatment of seizures. Two patients with SE and severe impairment of consciousness were treated with an intravenous valproate (ivVPA) bolus followed by continuous infusion. MEASUREMENTS AND RESULTS: In three cases, neurological and MRI abnormalities completely resolved in about a week. Another patient died due to subarachnoid hemorrhage. CONCLUSION: Posterior reversible encephalopathy syndrome is a well described clinical and neuroradiological syndrome characterized by headache, altered mental status, cortical blindness and seizures, and a diagnostic MRI picture; usually reversible, PRES can sometimes result in death or in irreversible neurological deficits, thus requiring early diagnosis and prompt treatment. PRES can have various etiologies, but pregnancy and postpartum more frequently lead to this condition. Treatment of seizures deserves special attention since the anti-epileptic drugs currently used in SE management may worsen vigilance as well as autonomic functions. Extensive research is needed to assess the role of ivVPA in this condition.
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5/42. Antegrade cerebral perfusion with hypothermic circulatory arrest: a case report.

    Techniques for the surgical correction of aortic aneurysms have steadily improved since the first described successful repair in 1955 by DeBakey et al. Despite these improvements, postoperative neurological complications remain a major factor in determining an adverse outcome. By using Deep Hypothermic Circulatory Arrest (DHCA), Retrograde Cerebral Perfusion (RCP) and now Selective Antegrade Cerebral Perfusion (SACP), the surgeon may provide better cerebral protection during extensive arch reconstruction. A 73-year-old female presented with an abnormal chest X-ray. Computerized tomography scan revealed a 4.5 cm mid aortic saccular arch aneurysm. Surgical intervention using cardiopulmonary bypass (CPB) with systemic cooling to 24 degrees C was employed. SACP was administered via cannulation of the innominate artery and the left common carotid artery using pediatric cannulae. Flow rates of 10 mL/kg/min and perfusion pressures of 60-90 mmHg were employed. Transcranial oximetry was used to monitor cerebral oxygen consumption. Circulatory arrest with SACP lasted for 36 min. Total bypass time was 178 min and myocardial ischemic time was 63 min. The patient was discharged on postoperative day five with no evident sequelae. While RCP has many benefits, SACP as used in this procedure may further improve patient outcome.
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6/42. Expanding the phenotypic spectrum of Lenz-Majewski syndrome: facial palsy, cleft palate and hydrocephalus.

    We report a sporadic case of Lenz-Majewski syndrome (LMS) with newly recognized manifestations including facial palsy, cleft palate and hydrocephalus developing later in infancy. The clinical course of the patient and neuroimaging studies are described. Increased intracranial pressure was recognized and treated early with the aim of preventing neurological morbidity.
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7/42. Intracranial plasma cell granuloma.

    We report two rare cases of primary intracranial plasma cell granuloma. The tumors probably arose from the dura and involved the cerebral parenchyma. These patients presented with clinical features of raised intracranial pressure and there was focal neurological deficit. The management issues are discussed.
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8/42. Anterior spinal decompression and fusion for cervical flexion myelopathy in young patients.

    OBJECT: The mechanism underlying cervical flexion myelopathy (CFM) is unclear. The authors report the results of anterior decompression and fusion (ADF) in terms of neurological status and radiographically documented status in young patients and discuss the pathophysiological mechanism of the entity. methods: Twelve patients underwent ADF in which autogenous iliac bone graft was placed. The fusion area was one segment in four cases, two segments in seven, and three segments in one. Neurological status, as determined by the Japanese Orthopaedic association (JOA) score, radiographic findings, and intraoperative findings were evaluated. The mean follow-up period was 63.3 months (range 20-180 months). Grip strength was significantly improved and sensory disturbances resolved completely. Intrinsic muscle atrophy, however, persisted in all patients at the final follow-up examination. Local kyphosis in the flexed-neck position at the fusion levels was corrected by surgery. Preoperative computerized tomography myelography revealed that the cord compression index, which was calculated by anteroposterior and transverse diameters of the spinal cord, decreased to 33 /- 6.2% in the flexed-neck position from 39.7 /- 9.9% in the extended-neck position. The anterior dura mater-spinal cord distance decreased to 1.9 /- 0.7 mm in the flexed-neck position from 4 /- 1.2 mm in extended-neck position. The posterior dura mater-spinal cord distance increased to 2.5 /- 1.1 mm in the flexed-neck position from 1.3 /- 0.5 mm in the extended-neck position. CONCLUSIONS: Postoperative neurological status was improved in terms of grip strength, sensory disturbance, and JOA score, and local kyphosis in the flexed-neck position at the fusion levels was reduced and stabilized by ADF. In most cases local kyphosis in the flexed-neck position was demonstrated at the corresponding disc level, as were cervical cord compression and decrease of the anterior wall of the dura mater-spinal cord distance in the flexed-neck position. Therefore, the contact pressure between the spinal cord and anterior structures (intact vertebral bodies and intervertebral discs) in the mobile and kyphotic segments was considered to contribute to the onset of CFM. The ADF-related improvement of the clinical symptoms, preventing kyphotic alignment in flexion and decreasing movement of the cervical spine, supports the idea of a contact pressure mechanism. Furthermore, short ADF performed only at the corresponding segments can preserve more mobile segments compared with posterior fusion. Thus, ADF should be the first choice in the treatment of CFM.
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9/42. Uncompacted lamellae as a feature of tomaculous neuropathy.

    Clinical findings suggested an inherited tendency to pressure palsies, but in this case without a family history. This was confirmed pathologically by the identification of a tomaculous neuropathy showing some atypical features including the presence of uncompacted lamellae in a high proportion of fibres.
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10/42. magnesium in primary care and preventive medicine: clinical correlation of magnesium loading studies.

    Diagnostic categories that are useful for describing patients with acute or organ failure disease are generally less useful labels for primary care and preventive medicine patients whose conditions are better described by signs and symptoms. Symptoms may be clues to ill health but are not often criteria for a major diagnostic labeling. In my practice I have used a computer-based medical record system that permits portrayal and comparison of symptom data to correlate varying degrees of retention of an intramuscular magnesium-loading study (IMMLS) with symptom patterns in 172 patients. The group of patients who show a paradoxical excretion of more Mg than they were given in the IMMLS are a distinctive group with lower blood pressures and significantly fewer digestive and skin symptoms, fewer symptoms of inflammation but more emotional symptoms than those with normal Mg excretion. The group who retained > 49% of the load had higher blood pressures and significantly fewer symptoms of inflammation of the skin and of the reproductive tract.
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