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CMT1X is caused by mutations in the connexin-32 gene located on chromosome Xq132,168,170,178 180 Connexins are gap junction structural proteins, which are important in cell-to-cell communication Connexin-32 oligomerizes into a hexameric structure on the Schwann cell lamella in the paranodal region and Schmidt Lanterman incisures, where it forms intercellular channels, which allow diffusion of ions, nutrients, and other small molecules through the compact myelin to the inner most layers of the myelin sheath and the axon itself The mutations in connexin-32 lead to demyelination and axonal degeneration176,181

If the parameters to an arithmetic operation are known at compile time (for example, using literals and constants), then the GCCE compiler will be able to flag the potential integer overflows GCCE being one of the compilers that targets the physical phone Each of these should be reviewed to ensure that the overflow does not cause an issue Note that the four SID and the four VID security policy macros will trigger a false positive integer overflow and can be safely ignored

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Muscular Atrophies, 6), except that it is inherited in an autosomal-dominant inheritance rather than X linked183 Muscle cramps are the earliest symptoms Individuals who are affected usually develop progressive proximal muscle atrophy, weakness, and fasciculations in the legs worse than in the arms after the age of 30 years (mean 45 +/ 6 years) People become nonambulatory 5 20 years after onset Facial muscles are also slightly weak, but neck exors and extensors remain relatively strong The tongue may be slightly weak but dysphagia and dysarthria are not common Nevertheless, bulbar and respiratory muscle weakness can develop late in the course of the disease Some patients complain of mild dysesthesias in the distal legs and hands Decreased sensation to all modalities, particularly vibratory preception and proprioception, is evident on examination As in Kennedy s disease, muscle stretch re exes are diminished or absent, neurogenic tremor is common, and there is an association with type 2 diabetes mellitus

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Serum creatine kinase (CK) levels are often mildly elevated SNAPs are reduced in amplitude or unobtainable as in Kennedy s disease CMAP amplitudes can be moderately decreased, while the distal latencies and NCVs are relatively preserved Needle EMG reveals diffuse fasciculation and brillation potentials and decreased recruitment of long-duration, large-amplitude, polyphasic MUAPs

Nerve biopsies show a loss of large and small myelinated nerve bers with preservation of unmyelinated nerve bers183 An autopsy demonstrated only a few remaining atrophic anterior horn cells along with signi cant loss off neurons in the spinal roots, cauda equina, and dorsal root ganglia183

SymbianOS has a system known as leaves and traps for handling many error conditions Calling User::Leave(TInt32 ErrorCode) is directly analogous to the throw statement from standard C++, and the TRAP/TRAPD macros take the place of the try/catch statements (The TRAP macro requires the developer to explicitly declare an error code variable, whereas the TRAPD macro does this automatically) A function that could potentially cause such an error is referred to as a function that may leave These can be identified in the standard library through a naming convention each function whose name ends with a capital L can potentially leave

CMT 2X is a rare X-linked recessive axonal motor and sensory neuropathy associated with deafness and mental retardation182 Distal limb weakness, atrophy, and sensory loss develop in early childhood Affected males often develop signi cant hearing loss and may also have mental retardation, while female carriers are asymptomatic CMT 2X links to Xq24 q25, although the speci c gene has not yet been identi ed

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This disorder has been linked to chromosome 3p141 q13, but the gene has not been identi ed (Table 9 1)183

Proximal hereditary motor and sensory neuropathy/ neuronopathy resembles Kennedy disease (see Spinal

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