TL;DR: MSA was considered a rare disease but many patients with MSA are misdiagnosed as suffering from idiopathic Parkinson's disease, it is clear from a large postmortem series of parkinsonian brains that cases of M SA are more common than previously thought.
Abstract: Multiple system atrophy (MSA) is a degenerative disease of the central nervous system. Dejerine and Thomas in 1900 were the first to use the term olivopontocerebellar atrophy (OPCA) in two sporadic cases,1 although Menzel described the first case in 1891. Shy and Drager in 1960 described four cases of a “neurological syndrome with orthostatic hypotension”.2They defined the full syndrome comprising of orthostatic hypotension, urinary and rectal incontinence, loss of sweating, iris atrophy, external ocular palsies, rigidity, tremor, loss of associated movements, impotence, atonic bladder, loss of rectal sphincter tone, fasciculations, wasting of distal muscles, evidence of a neuropathic lesion in the electromyogram suggesting involvement of the anterior horn cells, and the finding of a neuropathic lesion in the muscle biopsy. Adams et al first described a syndrome designated striatonigral degeneration in the early 1960s.3 These patients had mild autonomic failure and ataxia and were shown to have lesions in the olivopontocerebellar system. Graham and Oppenheimer first proposed the term multiple system atrophy in 1969 and suggested that OPCA, idiopathic orthostatic hypotension, the Shy-Drager syndrome, and striatonigral degeneration “are merely the expression of neuronal atrophy in a variety of overlapping combinations”.4
MSA is a sporadic disease. MSA was considered a rare disease but many patients with MSA are misdiagnosed as suffering from idiopathic Parkinson's disease. It is clear from a large postmortem series of parkinsonian brains that cases of MSA are more common than previously thought. Between 4% and 22% of the brains in parkinsonian brain banks have MSA.5 According to one estimate the prevalence of MSA is 16.4 per 100 000 population. Mean age of disease onset is 53 years (range 36–74) and the median disease duration to death is five years (range 1–11). Both sexes are affected equally.6
MSA can cause any combination …
TL;DR: The spectrum of essential iris atrophy began as a corneal endothelial degeneration, which, with ectopic endothelial membrane overgrowth over an open angle, followed by contraction of this membrane and further growth onto the iris, accounted for all aspects of the syndrome.
TL;DR: Patients with Chandler's syndrome had more severe corneal edema than the rest of the group, while those with progressive iris atrophy or the Cogan-Reese syndrome had worse secondary glaucoma.
Abstract: • Iridocorneal endothelial syndrome is generally considered to have three major variations: Chandler's syndrome, progressive (essential) iris atrophy, and the Cogan-Reese syndrome. To better understand the clinical significance of this classification, we studied the medical records of 37 consecutive patients from our practice, comparing the presentation and course of the three subgroups. Chandler's syndrome was the most common clinical variant within this spectrum of disease (21 cases). Patients with Chandler's syndrome had more severe corneal edema than the rest of the group, while those with progressive iris atrophy (8 cases) or the Cogan-Reese syndrome (8 cases) had worse secondary glaucoma.
TL;DR: The relative frequency of a dilated pupil, together with the common finding of focal iris atrophy after minimal surgical trauma to the iris in cases of keratoconus, forces one to conclude that the pathology in this condition is not confined to the cornea but probably extends to theIris and possibly to the scleral envelope as well.
Abstract: The present study reveals that pupillary abnormalities are common after keratoplasty for keratoconus and that, in addition to the fixed dilated pupils which we have found in 7.8 per cent. of eyes, varying degrees of partially dilated pupil frequently occur after operation. In our experience, glaucoma is not a sequel to the simple paretic pupil, a finding which confirms the results of the smaller series of Alberth and Schnitzler (1971); glaucoma thus seems to be no more a special complication of keratoplasty for keratoconus than it is of keratoplasty for any other corneal pathology. The paretic pupils can be explained on the basis of ischaemic atrophy of the sphincter pupillae muscle secondary to an iris strangulation phenomenon occurring during surgery in the manner we have discussed. The relative frequency of a dilated pupil, together with the common finding of focal iris atrophy after minimal surgical trauma to the iris in cases of keratoconus, forces one to conclude that the pathology in this condition is not confined to the cornea but probably extends to the iris and possibly to the scleral envelope as well.