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Cases reported "Curcuma"

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1/4. Central deafness in a young child with moyamoya disease: paternal linkage in a Caucasian family: two case reports and a review of the literature.

    A case of 'central deafness' is presented in a 3-year-old male Caucasian child with moyamoya disease (MMD); a rare, progressive and occlusive cerebrovascular disorder predominantly affecting the carotid artery system. documentation of normal peripheral auditory function and brainstem pathway integrity is provided by acoustic admittance, otoacoustic emission and brainstem auditory evoked potential measurements. The lack of behavioral response to sound, and absent middle and long latency auditory evoked potentials suggest thalamo-cortical dysfunction. magnetic resonance imaging showed diffuse ischemic damage in subcortical white matter including areas of the temporal lobes. In addition, there were multiple and focal cortical infarctions in both cerebral hemispheres, focused primarily in the frontal, parietal and temporal areas. Taken together, these structural and functional abnormalities in addition to severely delayed speech and language development are consistent with the diagnosis of central deafness and suggest a disconnection between higher brainstem and cortical auditory areas. The child's father also has MMD, but was diagnosed only recently. The presence of paternal linkage is informative since it rules out x-linked recessive and maternal inheritance. To our knowledge, this represents the first documented case of paternal linkage in MMD with central deafness in a Caucasian child with no apparent Japanese ancestry. Herein, we focus on central auditory dysfunction and consider how lesion-induced changes have contributed to a deficit in basic auditory responsiveness, including a severe disturbance in receptive and expressive auditory-based speech and language skills.
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2/4. Histopathology of the ears, eyes, and brain in Norrie's disease (oculoacousticocerebral degeneration).

    Norrie's disease is an x-linked recessive disorder characterized by progressive oculoacousticocerebral degeneration. The light and electron microscopic changes in the temporal bones, eyes, and brain of an affected 77-year-old man who suffered from bilateral profound sensorineural hearing loss, blindness, and mental retardation are described. The inner ears showed marked atrophy of the stria vascularis, severe degeneration of hair cells and cochlear neurons, and connective tissue proliferation in the spiral ganglion, osseous spiral lamina, and walls of the membranous vestibular labyrinth. The eyes showed detached retinae, dense proliferation of fibrillary glial cells in the retina and vitreous, severe atrophy of the optic nerves, and degenerative hyalinization of blood vessels. This case is the first published report of the histopathology of the inner ear in Norrie's disease.
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3/4. Palinacousis: a case report.

    Palinacousis (auditory perseveration) is a rarely reported symptom of temporal lobe dysfunction. We describe a new case. A 50-year-old woman presented with nausea, vomiting, and global dysphasia, followed by two generalized seizures. Examination was otherwise normal, and computed tomography showed a small area of enhancement near the left sylvian fissure; there was a left temporal focus on the electroencephalogram. Treatment with phenytoin was instituted, and speech improved, with residual fluent dysphasia. Three days postictally, the patient complained of "echoing voices" in her right ear. Words or fragments of sentences recently uttered by the patient or others were perceived to recur unaltered for minutes to hours. Sounds other than speech were also affected. One week later the voices had disappeared, but a ticking sound was present; this also faded subsequently. The palinacousis never recurred; the patient was later found to have a Grade IV astrocytoma of the left temporal lobe, which caused her demise 8 months later. The features of this case are similar to those previously reported and favor an epileptic etiology. Palinacousis should be recognized as a sign of organic temporal lobe disease and not confused with manifestations of psychotic illness.
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4/4. Lyme borreliosis--an unusual cause of vertigo.

    A total of 2055 consecutive vertigo patients were examined in a prospective study in an area endemic for Lyme borreliosis for clinical signs of Lyme borreliosis or serum antibodies against borrelia burgdorferi. Of these, 41 patients (2%) had positive levels of serum antibodies against B. burgdorferi. The incidence of seropositivity against B. burgdorferi among the vertigo patients did not differ from the incidence of the normal Finnish population. In addition to seropositivity the criteria used for Lyme borreliosis included previous erythema migrans, a positive polymerase chain reaction (PCR) or positive serum immunoblot. Eight patients were diagnosed as having Lyme borreliosis. This disease is a rare but possible cause of vertigo. Seropositivity alone is an insufficient finding for the diagnosis of Lyme borreliosis and should be supported by the clinical findings, the patient's history and other laboratory findings, such as immunoblotting or PCR. Although Lyme borreliosis seems to be a rare cause of vertigo, it must be kept in mind in the differential diagnosis of vertigo.
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Last update: April 2009
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