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Peripheral nerve biopsies in patients with the more common sensorimotor polyneuropathy demonstrates axonal degeneration and some degree of secondary segmental demyelination (Fig 14 1)1,2,6 Nonspeci c perivascular in ammation involving perineurial or endoneurial blood vessels is occasionally seen Rarely, necrotizing vasculitis is appreciated Biopsy of sensory nerves in patients with sensory neuronopathy/ganglionopathy demonstrated a loss of large myelinated bers and perivascular lymphocytic (CD8 T cells) in ammation involving endoneurial or perineurial vessels1 3 Biopsy of the dorsal root ganglion may reveal lymphocytic (mainly CD8 T-cells) in ltration and degeneration of cell bodies3 Reduced epidermal nerve ber density or abnormal morphology may be demonstrated on skin biopsies in a non-length-dependent manner, suggesting that patients with painful small ber neuropathies commonly have a small ber sensory neuronopathy/ganglionopathy rather than a dying-back axonopathy13
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1 Open Flash and create a new Flash file 2 Use the drawing tools in the Toolbar to draw a cloud on the Stage 3 Using the Selection tool, draw a selection area around the cloud to select it 4 Press F8 on your keyboard to convert the cloud to a symbol 5 Name the cloud mcCloud, choose Movie Clip for the type, and click
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while normal in the legs1 3,5,18 20 In addition, there may be asymmetric involvement Motor conduction studies and electromyography (EMG) are usually normal If the trigeminal nerve is affected, blink re exes are also abnormal21 An important clinical and electrophysiological feature that can help distinguish length-dependent sensory neuropathy from a sensory neuronopathy/ganglionopathy is the preservation of the masseter re ex or jaw jerk in the latter22 The masseter re ex is unique amongst the stretch re exes in that the cell bodies of the afferent limb lie in the mesencephalic nucleus within the CNS as opposed to the dorsal root ganglia where the sensory cell bodies innervating the extremities lie The Gasserian ganglia, which is responsible for conveying sensory nerves responsible for facial sensation and the blink re ex, also reside outside the CNS
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assigns the value 25 (since number has a value of 5) to the variable pointed to by squarePtr. This has the effect of assigning the value 25 to square. When SquareIt() returns control to main(), the value of square has been changed, as evidenced by the screen shot in Figure 7-9. If you d like to give this code a try, you ll find it in the Learn C Projects folder, inside the 07.02 squareIt subfolder.
The pathogenic basis of the distal sensory or sensorimotor polyneuropathy is unknown but is presumably autoimmune in nature Some cases may cause vasculitis The sensory neuronopathy/ganglionopathy appears to be the result of cell-mediated autoimmune attack directed against the sensory ganglia The speci c antigen(s) and trigger of the autoimmune attack are not known
There is no speci c treatment for neuropathies related to Sj gren syndrome When vasculitis is suspected, o immunosuppressive agents may be bene cial Occasionally, the sensory neuronopathy/ganglionopathy stabilizes or improves with immunotherapy such as IVIG3,11,18,23,24
The default registration for symbols is top left Top left is also the X/Y origin on the Stage To keep things consistent, it is usually best to give all symbols top-left registration I will discuss exceptions to this later on in this book
Figure 7-9. The results of running squareIt. This program shows how to pass a parameter by address.
Peripheral neuropathy occurs in at least 50% of patients with rheumatoid arthritis (RA) Vasculitic neuropathy develops in 40 50% of patients with RA, making it the third most common cause of vasculitic neuropathy following polyarteritis nodosa (PAN) and isolated peripheral nervous system vasculitis12,25 28 Symptoms usually manifests 10 15 years after manifestations of other symptoms of RA, although rarely the neuropathy can be the presenting feature27 Rheumatoid vasculitis can present with a mononeuropathy multiplex, overlapping mononeuropathy multiplex, or generalized symmetric pattern of involvement Neuropathies may also be due to drugs used to treat the RA (eg, tumor necrosis blockers and le unomide)
Figure 14 2 Rheumatoid arthritis Sural nerve biopsy reveals an epineurial vessel perivascular in ammation and scattered perineurial and endoneurial dilated capillaries with thickened walls Paraf n section stained with Hematoxylin & Eosin (H&E)
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