Background: Juvenile muscular atrophy of the distal upper extremity (Hirayama disease) is characterised by anterior horn cell loss in the lower cervical cord , presumably caused by anterior displacement of the dural s...Background: Juvenile muscular atrophy of the distal upper extremity (Hirayama disease) is characterised by anterior horn cell loss in the lower cervical cord , presumably caused by anterior displacement of the dural sac during neck flexio n. A recent report suggests that atopy and IgE may contribute to anterior horn d amage. Abstract Objective: To investigate whether IgE is a contributing factor in Hirayam a disease. Methods: Serum total IgE and allergen specific IgE were examined in 2 0 consecutive patients, and their correlations with clinical profiles investigat ed. Results: Past or present history of allergy/atopy was found in only four pat ients (20% ), but serum IgE was raised in 14 (70% ). Patients with hyperIgEaem ia had more severe clinical disabilities than those without (p = 0.01). In patie nts whose history of Hirayama disease was less than five years, serum total IgE was higher than in those with the disease for five years or more (p = 0.05). Con clusions: The results suggest that hyperIgEaemia is often associated with Hiraya ma disease and can facilitate its pathophysiology, particularly in the early pha ses of the disease. HyperIgEaemia does not appear to involve the anterior horn c ells primarily.展开更多
Putaminal hemorrhage presenting pure sensory stroke is rare.We describe a case of left putaminal hemorrhage presenting contral ateral hemisensory disturbance without hemiparesis. A 52-year-old man developed analgesia ...Putaminal hemorrhage presenting pure sensory stroke is rare.We describe a case of left putaminal hemorrhage presenting contral ateral hemisensory disturbance without hemiparesis. A 52-year-old man developed analgesia and thermoanesthesi a in the right half of his body, but deep sensation was relatively well preserve d. Neuroradiological and somatosensory evoked potential findings suggested that thalamocortical sensory pathways to the secondary somatosensory cortex (S2)were involved, whereas those to the primary somatosensory cortex(S1) were spared. In experimental animals, spinothalamic projections from the thalamic nucleus input directly to S2. In humans, thalamocortical pathways are still a subject of debat e,but results of recent functional imaging studies suggest that the pathway of p ain inputs directly to S2 and that of tactile sensation to S2 via S1. Our findin gs support these reports.展开更多
文摘Background: Juvenile muscular atrophy of the distal upper extremity (Hirayama disease) is characterised by anterior horn cell loss in the lower cervical cord , presumably caused by anterior displacement of the dural sac during neck flexio n. A recent report suggests that atopy and IgE may contribute to anterior horn d amage. Abstract Objective: To investigate whether IgE is a contributing factor in Hirayam a disease. Methods: Serum total IgE and allergen specific IgE were examined in 2 0 consecutive patients, and their correlations with clinical profiles investigat ed. Results: Past or present history of allergy/atopy was found in only four pat ients (20% ), but serum IgE was raised in 14 (70% ). Patients with hyperIgEaem ia had more severe clinical disabilities than those without (p = 0.01). In patie nts whose history of Hirayama disease was less than five years, serum total IgE was higher than in those with the disease for five years or more (p = 0.05). Con clusions: The results suggest that hyperIgEaemia is often associated with Hiraya ma disease and can facilitate its pathophysiology, particularly in the early pha ses of the disease. HyperIgEaemia does not appear to involve the anterior horn c ells primarily.
文摘Putaminal hemorrhage presenting pure sensory stroke is rare.We describe a case of left putaminal hemorrhage presenting contral ateral hemisensory disturbance without hemiparesis. A 52-year-old man developed analgesia and thermoanesthesi a in the right half of his body, but deep sensation was relatively well preserve d. Neuroradiological and somatosensory evoked potential findings suggested that thalamocortical sensory pathways to the secondary somatosensory cortex (S2)were involved, whereas those to the primary somatosensory cortex(S1) were spared. In experimental animals, spinothalamic projections from the thalamic nucleus input directly to S2. In humans, thalamocortical pathways are still a subject of debat e,but results of recent functional imaging studies suggest that the pathway of p ain inputs directly to S2 and that of tactile sensation to S2 via S1. Our findin gs support these reports.
