spinal muscular atrophy, distal, congenital non-progressive

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spinal muscular atrophy, distal, congenital non-progressive

A clinically variable neuromuscular disorder (OMIM:600175) characterised by congenital motor neuron disorder restricted to the lower body.

Clinical findings
Non-progressive thigh muscle atrophy, weak thigh adductors and knee and foot extensors, minimal jaw muscle and neck flexor weakness, flexion contractures of knees and pes equinovarus. Tendon reflexes are normal.

Molecular pathology
Caused by defects in TRPV4, which encodes a nonselective cation channel thought to sense mechanical and osmotic changes and regulate systemic osmotic pressure.
References in periodicals archive ?
First, an experimenter described the task, then provided a demonstration of the DSMAC program using a choice situation familiar to participants but different from the experimental choice task.
Second, subjects were directed to begin DSMAC on the computer in front of them, which began by asking them first to perform a Q-sort of the 25 camera features, then to assign weights to each on a scale having a range of 1-100, with 100 being the most important.
Sequential decision-making strategies of expert and novice consumers
The Block II used in Desert Storm demonstrated extreme accuracy, due to Dsmac (digital scene-matching area correlation) navigation in the terminal phase, but required lengthy planning.
Far reaching propositions: sea-based, barrel-launched guided projectiles and conventionally armed cruise missiles are already demonstrating considerable effectiveness. The same is true of land-based weapons. Several countries are now working to exploit the full potential of such weapon systems for the precision engagement of critical targets at the beachhead, on the extended battle-field and far beyond. (Complete Guide)
Tomahawks navigated by terrain-contour matching (Tercom) with digital scene-matching area correlation (Dsmac) in the terminal phase, while the Calcms used less accurate GPS/INS.
Responding to criticism of lengthy planning, in April 1993 the first Block III Tomahawks were delivered with GPS receivers, allowing them to approach the target from any direction, and requiring only one terminal Dsmac scene for accuracy.
Missiles on a Cruise
Equipped with moderncruise missile technology of Terrain Contour Matching (TERCOM) and DigitalScene Matching and Area Co-relation (DSMAC), it can carry both nuclear and conventional warheads.
Pakistan testfires Cruise Missile Hatif-V11
The army said: "It also incorporates the most modern cruise missile technology of Terrain Contour Matching (TERCOM) and Digital Scene Matching and Area Co-relation (DSMAC), which enhances its precision and effectiveness manifolds."
Pakistan tests nuclear-capable Hatf VII cruise missile
This electro-optical system is called DSMAC (Digital Scene Matching Area Correlator) and it compares the image of the target area obtained by an infrared sensor in the missile's nose with a thermal image of the target under attack stored in the onboard computer before launch.
The US Navy's BGM-109 Tomahawk cruise missile; the problem weapon for arms control negotiators
The army said: "It also incorporates the most modern cruise missile technology of Terrain Contour Matching (TERCOM) and Digital Scene Matching and Area Co-relation (DSMAC), which enhances its precision and effectiveness manifolds." The test will consolidate Pakistan's strategic deterrence capability, and further strengthen national security.
Pakistan tests nuclear-capable Hatf VII cruise missile
According to ISPR here, it also incorporates the most modern cruise missile technology of Terrain Contour Matching (TERCOM) and Digital Scene Matching and Area Co-relation (DSMAC), which enhances its precision and effectiveness manifolds.
Pakistan conducts successful test fire of Cruise Missile Hatf-VII(Babur)
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