Cases reported "Cerebellar Diseases"

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1/5. Therapeutic effectiveness of acetazolamide in hindbrain hernia headache.

    A 43-year-old man had been suffering from exertional headache for 10 years. Sagittal sections on magnetic resonance imaging showed a Chiari type 1 malformation and a cerebellar arachnoid cyst. This syndrome, named hindbrain hernia headache, disappeared with oral acetazolamide. This treatment should be tried in patients with hindbrain hernia headache prior to considering surgical decompression of the foramen magnum.
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keywords = hindbrain
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2/5. Fusion of the cerebellar hemispheres ventral to the brainstem: a rare hindbrain-related malformation.

    INTRODUCTION: New variations of cerebellar malformations are being increasingly recognized using modern neuroimaging techniques. Until now only dorsal cerebellar fusion syndromes, such as the rhombencephalosynapsis and its variations have been recognized. CASE REPORT: We report on a 17-month-old male infant presenting with an exceptional hindbrain related malformation characterized by midline fusion of the cerebellar hemispheres ventral to the brainstem and causing symptoms due to brainstem compression. DISCUSSION AND CONCLUSION: A more detailed understanding of the cerebellar embryogenesis is required to unravel the underlying mechanisms leading to this type of cerebellar malformation, which cannot easily be integrated into the common classification systems. Both the morphological features and the clinical presentation are different from those of other cerebellar structural abnormalities. If this type of congenital malformation is detected more frequently in the future, it seems reasonable that it should be added to the list of cerebellar malformations as a distinct type.
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keywords = hindbrain
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3/5. Subtorcular occipital encephaloceles. Anatomical considerations relevant to operative management.

    Three cases of occipital encephalocele, one with associated myelomeningocele, are presented. All received preoperative evaluation with magnetic resonance imaging. Such studies provide optimal demonstration of the cerebral and hindbrain anatomy to guide operative treatment and formulate prognosis. review of available radiographic, operative, and pathological information suggests that most, if not all, occipital encephaloceles are associated with an anomaly of the hindbrain, and the usual anomaly is a rhombic roof encephalocele. In such cases, the site of cranial herniation is caudal to the torcula, regardless of the presence or absence of occipital lobe tissue within the sac. Experimental and clinical analysis suggests that occipital encephaloceles most likely arise from abnormalities in the development of the skull base.
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keywords = hindbrain
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4/5. syncope as a presenting feature of hindbrain herniation with syringomyelia.

    syncope is rare as a presenting symptom of syringomyelia. Three cases are described in which syncope brought the patient to hospital and in each case syringomyelia was subsequently diagnosed and treated. The suggestion is made that impaction of hindbrain hernia or the Chiari malformation may be a causative mechanism of loss of consciousness in such cases; the three examples presented all did well after posterior fossa surgery to decompress the hindbrain hernia.
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keywords = hindbrain
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5/5. Chiari I "malformations"--an acquired disorder?

    Caudal herniation of the hindbrain, indistinguishable from the Chiari I deformity, may occur after the establishment of spinal subarachnoid shunts and become symptomatic years after the procedure. Examples are presented and others are cited from the literature. It is proposed that the force responsible for the displacement is the difference in pressure between the cranial and spinal compartments. On the basis of these observations and other considerations as well, a similar process, disproportionate absorption of cerebrospinal fluid from the spinal region, might account for the spontaneous form of the Chiari I deformity.
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keywords = hindbrain
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