Acute Idiopathic Blind Spot Enlargement AIBSE

Acute idiopathic blind spot enlargement (AIBSE) was described in 1988 in a report including seven patients that presented a peripapillary scotoma producing symptomatic enlargement of the blind spot objectively identified by visual field testing. They were all young patients aged from 25 to 39 years with a 5/2 female predominance and 2/7 patients had previous episodes

[29]. Visual acuity, colour vision, pupillary responses, funduscopy and fluorescein angiogra-phy were all normal. The only additional abnormal finding was an abnormal focal ERG indicating retinal dysfunction around the optic disc at the origin of the visual field defect. Most probably AIBSE and MEWDS are the same disease with the exception of the usual fundus findings that were not found in this and other reports on AIBSE. As indicated by Hamed et al., the retinal lesions may already have subsided at the time of examination or were subclinical

[30]. If ICGA had been available and performed by the authors of this and subsequent reports, it is probable that AIBSE would never have been described as a separate entity, as ICGA is presently the method of choice for diagnosing atypical MEWDS in patients consulting at a later stage of the disease or presenting subclinical disease [31]. The numerous reports on associated blind spot enlargement in many of the diseases presently regrouped in the category of primary inflammatory choriocapillaropathy are an indication for a common physiopathogenic mechanism [32-35]. Furthermore, the reports including ICGA investigations in their work show that visual field alterations are related to peripapillary hypofluorescence, indicating choriocapillaris non-perfusion as the phys-iopathogenic process at the origin of blind spot enlargement [31,36,37]. This pathologic characteristic, found in many of the PICCPs, seems to indicate a common weakness of the peripapil-lary choriocapillaris, possibly more sensitive to closure secondary to inflammation, with subsequent outer retinal ischaemia and dysfunction producing a peripapillary scotoma [35].

Multiple Evanescent White Dot Syndrome

(MEWDS) and Acute Idiopathic Blind Spot

Enlargement (AIBSE)

• Viral flu-like syndrome often found in preceding fortnight in up to 60% of cases

• Usually unilateral and unique episode

• Symptoms: photopsias, scotomata, visual loss that can be severe in some cases

• Visual loss found in >90% of cases: drop in visual acuity from slight to severe, visual field changes and blind spot enlargement

• Fundus findings: discrete discolorations in midperiphery and granular aspect of macula

• ICGA: hypofluorescent dots and peripapillary hypofluorescence in acute phase resolving in 4-8 weeks

• FA: hypofluorescence (early), discretely hyperfluorescent foci (late) or absent FA findings ± cystoid macular edema; disc hyperfluorescence

• Spontaneous resolution of signs and symptoms within 6-10 weeks in ± all cases

• AIBSE: entity described before the ICGA era behaving as MEWDS without fundus signs that have probably resolved or are subclinical

Acute Posterior Multifocal Placoid Pigment Epitheliopathy (APMPPE) or Acute Multifocal Ischaemic Choriocapillaropathy (AMIC)

Acute posterior multifocal placoid pigment epitheliopathy (APMPPE) was first described by Donald Gass in 1988 in a report of three female patients aged 19,22 and 22 years, showing rapid loss of central vision associated with multifocal, yellow-white placoid lesions occurring in one eye, with sequential involvement of the second eye, followed by resolution of the fundus lesions and visual improvement over weeks or months thereafter [38]. The hypothesis that was put forward was an acute cellular response on the part of the pigment epithelium to a local noxious agent. Deutman and colleagues, based on the choriocapillaris non-perfusion on early FA frames, clearly indicated that it was the chorio-capillaris rather than the pigment epithelium that was primarily involved and he suggested renaming the disease acute multifocal is-chaemic choroidopathy (AMIC) [9,10]. As for other PICCPs, APMPPE/AMIC is preceded by a febrile, infectious or flue-like episode in at least half of the patients if a careful history is taken [28,39].It was associated with diverse infectious episodes preceding the ocular involvement such as mumps, streptococcal group A infection and a hepatitis vaccination [i7-i9].A clinical picture identical to APMPPE was seen in a patient with Lyme disease and a patient with secondary syphilis with resolution of the acute lesions following treatment of the underlying cause [40, 41]. Like other PICCPs,APMPPE is predominant in young individuals that are in the 2nd-4th decade of life. Concerning disease mechanism, there is an appreciable body of information and evidence that the lesional process in APMPPE is a vasculitis. On the one hand, there is a report of systemic vasculitis that produces a clinical picture exactly similar to APMPPE [42]. On the other hand, there are many reports of APMPPE associated with a vasculitis elsewhere, in the cerebellum, with associated nephritis and meningoencephalitis [43-47].

Summary for the Clinician

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