Gene/Protein Disease Symptom Drug Enzyme Compound
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A 68-year-old man presented with right eye pain and vertigo. Thereafter, he gradually leaned rightward, then laid down. He felt nausea and vomited. His right upper eyelid drooped and he felt dysethesia of the right hand. On neurological examination, ptosis of his right eye with slightly miotic right pupil, paresis of the right soft palate and hoarseness were noted. Arm deviation test demonstrated rightward deviation. He presented sensory ataxia of the right upper and lower extremities: finger nose test showed mild dysmetria of the right upper extremity, heel knee test demonstrated dysmetria of right lower extremity and these findings worsened when he closed his eyes. He showed mild bending of his bilateral ring and little fingers when he did rapid alternative movement. He leaned rightward when he sat and closed his eyes. Position sense of his right upper and lower extremities was decreased and sometimes he could not answer correctly when asked on which direction his finger pointed. Pinprick sensation was mildly decreased on the left side not including the face. Touch and vibration sense were normal. SEP findings on upper and lower extremity stimulation were normal. MRI of the brain showed T2 high intensity and partially T1 low intensity lesion at the right medulla (Figure). MR angiography showed no apparent lesion of major arteries such as dissection of the vertebral arteries. He complained and presented with hiccup initially. On MRI, the lesion was thought to involve the spinothalamic tract, medial lemniscus and inferior olivary nucleus. Ambiguus nucleus was in the lesion and solitary nucleus near the lesion. There is no report that seems to describe clinical features of a lesion like that in this case. Intermediate medullary infarction may present dissociated sensory disturbance like Brown-Sequard syndrome and position sensory disturbance without disturbance of vibration sense.
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PMID:[Intermediate medullary infarction: a case report]. 1609 22

A 42-year-old woman suddenly developed weakness in her left extremities when stretching her neck two days after the onset of a nuchal pain. Because computed tomography (CT) of the brain did not show any apparent lesion, the patient had initially been treated as having a cerebral infarction until magnetic resonance imaging (MRI) of the cervical spine revealed a presence of a cervical epidural hematoma the next day. She was therefore transferred to our hospital, and a neurological examination showed moderate left hemiparesis, dissociated sensory loss under C6 on the right side, urinary incontinence, and left miosis and ptosis. A CT of the cervical spine demonstrated an anteriorly located left-sided epidural hematoma extending from C4 to C7. The T2-weighted MRI revealed hyperintense lesions around the gray matter on the left side that were compressed by the epidural hematoma. The patient underwent an emergent laminoplasty from C3 to C7. Although her neurological signs were consistent with Brown-Sequard syndrome, which was associated with left-sided Homer's sign, they gradually and completely subsided following surgery. The authors therefore emphasize that cervical lesions should be considered in the differential diagnosis in patients with acute onset of hemiparesis.
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PMID:[Spontaneous cervical epidural hematoma presenting with hemiparesis following neck extension: a case report]. 1870 May 37

Idiopathic spinal cord herniation is a rare spinal cord disorder caused by spinal cord prolapse through a adural defect. It is a curable disease, so early detection is of particular importance. We report a 38-year-old woman with Brown-Sequard syndrome which was caused by the thoracic spinal cord herniation. Her weakness was almost completely resolved after surgical management, which emphasizes the importance of early diagnosis and surgical management in this rare disease entity.
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PMID:Idiopathic spinal cord herniation as a treatable cause of progressive brown-sequard syndrome. 1951 34