Cases reported "Blindness, Cortical"

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1/3. subacute sclerosing panencephalitis: a cause of acute vision loss.

    A 4-year-old male presented with only acute vision loss. His neurologic examination, funduscopic examination, and pupils were normal. Cranial magnetic resonance imaging revealed abnormal hyperintense, bilaterally symmetric lesions (on T(2)-weighted and fluid-attenuated inversion recovery, images) in bilateral optic radiations, pulvinar region in the thalami, crus posterior of internal capsules, periventricular white matter, and unilaterally left anterior pons. Elevated measles antibody titers in the cerebrospinal fluid confirmed the diagnosis of subacute sclerosing panencephalitis. Vision loss improved and cranial magnetic resonance imaging findings regressed,but myoclonic jerks and deterioration began 7 months later. The diagnosis of subacute sclerosing panencephalitis should be considered in cases with acute vision loss resulting from cortical blindness even when classical findings of the central nervous system do not exist.
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2/3. Obstetrical anaesthesia for a parturient with preeclampsia, hellp syndrome and acute cortical blindness.

    PURPOSE: To report the management of a multigravida presenting with preeclampsia, hellp syndrome and acute cortical blindness for Caesarean section. CLINICAL FEATURES: A 39-yr-old woman, with three past uncomplicated pregnancies presented at 33 wk with acute cortical blindness. Based on clinical and laboratory assessment, a diagnosis of preeclampsia with hellp syndrome was made. A CT scan of her head demonstrated ischaemic lesions of her basal ganglia, extending superiorly to involve both posterior parietal and occipital regions. Infusions of magnesium sulphate and hydralazine were started and an urgent Caesarean section was performed under subarachnoid anaesthesia after insertion of an arterial line and intravenous hydration. The course of anaesthesia and surgery was uneventful and she delivered a live 1540 g female infant. By the following morning, she had recovered some vision and visual recovery was complete by 72 hr postpartum. She underwent an MRI with angiography on the first postpartum day. Ischaemic lesions were confirmed in the same sites identified on CT scan but all major cerebral vessels were patent and no significant vascular abnormality was noted. Her postoperative course was uneventful and she was discharged home seven days postpartum after being prescribed labetalol for continued hypertension. CONCLUSION: The anaesthetic management of a parturient with acute cortical blindness and hellp syndrome is modeled on the underlying preeclamptic condition. Invasive monitoring is not routinely indicated but is specifically indicated in some cases. Provided that it is not contraindicated because of prohibitive risk to the mother, regional anaesthesia has particular advantage in these patients. In particular, the use of spinal anaesthesia, which has been discouraged by some for this patient population, should be re-evaluated.
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3/3. Childhood melas syndrome presenting with seizure and cortical blindness: a case report.

    An 11-year-old boy presented with seizure and cortical blindness. A T1 weighted magnetic resonance image of the brain showed high signal intensity in the bilateral corpus striatum and long T1 and T2 changes in the bilateral occipital and cerebellar hemispheric regions. Increased cerebrospinal fluid lactate concentration of 56.7 mg/dl and blood lactate concentration of 34.2 mg/dl were also noted. A muscle biopsy obtained from the quadriceps femoris muscle showed the presence of ragged red fibers and mitochondrial dna (mtDNA) analysis showed an A-->G mutation at nucleotide position 3243. MtDNA analysis of the patient's mother revealed the same mutation. These findings indicated melas syndrome (mitochondrial encephalomyopathy, lactic acidosis and stroke-like episodes).
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