Cases reported "Atrophy"

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1/6. Lower motor neuron disease associated with myelofibrosis.

    We present a patient who has signs pointing to the involvement of lower motor neurons and myelofibrosis. To our knowledge, unlike lymphoproliferative disorders, co-occurrence of myelofibrosis and lower motor neuron disease (MND) has not been reported so far. A 64-year-old male patient was admitted to our hospital with the complaint of painful cramps in his neck and forearms. On physical examination marked hepatomegaly and splenomegaly were found. On neurologic examination nasal quality of the voice and slight palatal weakness were detected. There were generalised slight weakness and atrophy in both proximal and distal muscle groups. Fasciculations were observed especially in forearm muscles and it was observed that he had been avoiding head movements because of painful muscle cramps. Deep tendon reflexes were hypoactive. Nerve conduction studies were normal. By needle electromyography, giant motor unit action potentials (amplitudes up to 8 mV), fibrillation potentials, positive sharp waves and fasciculation potentials were detected in all muscles which were investigated. A hypercellular bone marrow (100%) was determined by bone marrow biopsy. In addition to increased production of the myeloid and megakaryocytic lines, abnormal aggregation and grouping of megakaryocytes were seen. Reticular fibers were increased. He had some benefit of dyphenilhydantoin treatment given for the painful cramps in his neck and forearm muscles. hydroxyurea treatment was started for myelofibrosis. Six months later, his general condition was better, and the painful cramps were completely resolved. No marked deterioration has been detected in neurologic examination and electromyography for 1 year.
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keywords = voice
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2/6. Hemispheric dominance for emotions, empathy and social behaviour: evidence from right and left handers with frontotemporal dementia.

    Although evidence from primates suggests an important role for the anterior temporal cortex in social behaviour, human research has to date concentrated almost solely on the orbitofrontal cortex and amygdala. By describing four cases of the temporal variant of frontotemporal dementia we show how this degenerative condition provides an excellent model for investigating the role of the anterior temporal lobe, especially the right, in emotions, empathy and social behaviour. Assessments of semantic memory, processing of emotional facial expression and emotional prosody were made, empathy was measured, and facial expressions of emotion were coded. Of the two right handers described, one subject with predominantly left temporal lobe atrophy had severe semantic impairment but normal performance on all emotional tasks. In contrast, the subject with right temporal lobe atrophy showed severely impaired recognition of emotion from faces and voices that was not due to semantic or perceptual difficulties. empathy was lost, interpersonal skills were severely affected and facial expression of emotion was characterized by a fixed expression that was unresponsive to situations. Additionally, two left handers with right temporal lobe atrophy are described. One demonstrated the same pattern of hemispheric lateralization as the right handers and had emotional impairment. The other left hander showed the opposite pattern of deficits, suggesting a novel presentation of anomalous dominance with reversed hemispheric specialization of semantic memory and emotional processing.
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keywords = voice
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3/6. Thyroplasty type I (lateral compression) for dysphonia due to vocal cord paralysis or atrophy.

    Based on the experimental results of thyroplasty, thyroplasty type I which aims at medical shifting the vocal cord was performed on 8 patients with dysphonia, 6 with vocal cord paralysis and 2 with vocal cord atrophy. The surgery was conducted on either in- or out-patient basis and local anesthesia was used. Usually, a rectangular incision was made on the thyroid cartilage at the level of the vocal cord, and the fragmemt was depressed inward. A cartilage piece taken from the opposite side was used as a wedge, if necessary, to enhance the effect of lateral compression of the vocal cord. The voice after surgery was generally satisfactory, except in one case of traumatic vocal cord paralysis. Complications such as stridor or dyspnea were nil. As surgical intervention inside the thyroid cartilage is minimal, fine and reliable adjustment of depression is possible during the surgery.
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ranking = 1
keywords = voice
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4/6. Slowly progressive defect in recognition of familiar people in a patient with right anterior temporal atrophy.

    We report the case of a patient (C.O.) who showed a selective defect in the recognition of familiar people, with very mild disease progression during a period of 30 months resulting from focal atrophy of the right temporal lobe. On formal neuropsychological testing, C.O. obtained high scores on tests of general intelligence, episodic memory, language, executive functions, selective attention, visual recognition and visual-spatial abilities. On more specific tasks of familiar and unfamiliar face recognition, C.O. scored above the controls' means on perceptual tests, but obtained highly pathological results on amnesic-associative tests. His disorder of recognition of familiar people was not due to loss of person-specific information, since he obtained highly abnormal naming scores when presented with photographs of famous people but borderline or mildly abnormal scores in a tasks in which he had to name celebrities from verbal definitions. On the other hand, C.O.'s recognition disorder could not be considered to be a form of 'associative prosopagnosia' since a similar defect was observed when he was requested to access information about famous persons through their voice rather than their face. Two alternative interpretations are advanced to explain C.O.'s inability to access his relatively spared person-specific knowledge not only through the person's face but also through the person's voice. The first hypothesis is that, before accessing the person-specific information, unimodal recognition channels must converge into a multimodal, non-verbal person-recognition system and that the right anterior temporal cortices play a crucial role in this integrative activity. The second hypothesis is that the face-recognition units have privileged access to person-specific semantic knowledge and that other recognition subsystems require coactivation of the face-recognition units in order to access person-specific semantic information.
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ranking = 2
keywords = voice
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5/6. Spasmodic dysphonia combined with insufficient glottic closure by phonation.

    The authors describe the case history of a patient who suffered from symptoms deriving from two different origins. The patient's voice was spasmodic dysphonia-like interrupted and pressed. At the same time, his voice was powerless, too. The reason for this was that besides the spasmodic dysphonia caused by hyperkinesis, an incomplete closure of the vocal cords during phonation in the middle third was present. It was caused by the atrophy of the vocal cords. In order to eliminate the symptoms, initially we injected 25 IU Botox into the left vocal cord transcutaneously under the direction of EMG control. It resulted in a fluent, though breathy voice. In order to manage the closing insufficiency during phonation, we performed lipoaugmentation on the left vocal cord under high-frequency jet anaesthesia. The result of the two-step procedure was a fluent and clear voice. The speech without interruption lasted for 5 months, until the drug was eliminated. Of course, to prolong the result, the Botox injection should be repeated.
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keywords = voice
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6/6. Migration of the anterior segment following anterior commissure advancement: a case report.

    A 53-year-old man with severe vocal fold atrophy underwent bilateral type 1 thyroplasty and anterior commissure advancement. Postoperatively, he developed a strained voice with less projection and volume than prior to surgery. This was verified by objective assessment of vocal function. Videoendoscopy revealed bilateral false vocal fold fullness and blunting of the anterior commissure. magnetic resonance imaging demonstrated cephalic migration of the posterior ends of the implants and retrusion of the anterior commissure segment. Surgical exploration revealed that the type 1 implants had rotated and buckled. The anterior commissure segment was rotated and displaced inferiorly, and its inferior surface was tethered to the cricoid by scar tissue. The implants were removed, the anterior segment was repositioned and rigidly fixed, and bilateral lipoinjection performed. Vocal function was significantly improved, and endoscopy revealed normal tension and length of the vocal folds and restoration of the anterior commissure. This case demonstrates the importance of stable fixation during laryngeal framework surgery.
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ranking = 1
keywords = voice
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