PMCCPMCCPMCC

Search tips
Search criteria 

Advanced

 
Logo of bmjcrInstructions for authorsCurrent ToCBMJ Case Reports
 
BMJ Case Rep. 2010; 2010: bcr0820092219.
Published online Oct 12, 2010. doi:  10.1136/bcr.08.2009.2219
PMCID: PMC3029921
Rare disease
Initial neuro-ophthalmological manifestations in Churg–Strauss syndrome
Anne-Evelyne Vallet,1 Adrien Didelot,1 Fitsum Guebre-Egziabher,2 Martine Bernard,3 and François Mauguière1
1Department of Functional Neurology, Hopital Neurologique, Bron Cedex, France
2Department of Nephrology, Edouard Herriot Hospital, Lyon Cedex, France
3Department of Neuro-Ophthalmology, Hopital Neurologique, Bron Cedex, France
Correspondence to Anne-Evelyne Vallet, anne-evelyne.vallet/at/chu-lyon.fr
Churg–Strauss syndrome (CSS) is a systemic vasculitis with frequent respiratory tract involvement. It can also affect the nervous system, notably the optic tract. The present work reports the case of a 65-year-old man diagnosed as having CSS in the context of several acute onset neurological symptoms including muscle weakness and signs of temporal arteritis, including bilateral anterior ischaemic optic neuropathy (ON). Electroretinograms (ERGs) and visual evoked potentials (VEPs) were performed. Flash ERGs were normal whereas VEPs were highly abnormal, showing a dramatic voltage reduction, thus confirming the ON. The vision outcome was poor. Ophthalmological presentations of CSS have rarely been reported, but no previous case of sudden blindness documented by combined ERG and VEP investigations were found in the literature. The present case strongly suggests that the occurrence of visual loss in the context of systemic inflammation with hypereosinophilia should lead to considering the diagnosis of CSS.
Churg–Strauss syndrome (CSS), or allergic granulomatous angiitis, is a rare disease affecting small-sized to medium-sized vessels.1 2 The most frequent clinical manifestations are respiratory tract involvement (75%) such as asthma, which is an almost universal feature, pulmonary infiltrates and allergic rhinitis, followed by mononeuritis multiplex (65% to 75%), gastrointestinal, cardiac, dermatological and renal involvement.1 Neurological manifestations other than mononeuritis multiplex are uncommon.2 Ocular manifestations are rarely reported.3 We report the case of a patient diagnosed as having CSS after a series of neurological symptoms and signs including muscle weakness and temporal arteritis. He experienced sudden blindness caused by bilateral ischaemic optic neuropathy (ON) documented by electroretinographic (ERG) recordings and visual evoked potentials (VEPs).
Only a few cases of ischaemic ON in this context have been reported in the literature.410 To date no case documented by combined ERGs and VEPs has been published.
During a trip to Kenya, a 65-year-old man experienced unusually profuse rhinorrhoea, then, a few days later, a fever with intense proximal myalgia. He also described an episode of mandibular claudication followed by left periorbital pain, diplopia and blurred vision. These symptoms spontaneously resolved but the patient consulted a week after his return to France because of a global motor weakness predominating on his right side, in the context of recurring proximal myalgia, cephalalgia, fever and asthaenia.
He had a history of late-onset asthma since the age of 50. This was responsive to inhaled corticosteroid treatment and associated with an asymptomatic and moderate hypereosinophilia (1 g/litre) that had been discovered during a routine check-up 2 years before admission. He had travelled many times to Kenya since his retirement at the age of 60.
Following his consultation, routine blood tests showed marked hypereosinophilia (6.7 g/litre) associated with a systemic inflammatory response, and in September 2007 the patient was admitted to the Department of Infectious Diseases on suspicion of tropical parasitic disease. At 2 days after admission, the patient suddenly developed blurred vision, initially on the right and then bilaterally, and became blind within 24 h.
The patient was then transferred to the Neurology department. On admission he presented with a global muscle weakness clearly lateralised on his right side and predominant on the proximal muscle groups associated with diffuse myalgia, normal tendon reflexes and plantar reflexes flexor on both sides. The patient had recent paraesthesiae in the left median nerve territory without somatosensory deficit on clinical examination. He was hyperthermic (40 °C) but showed no signs of meningitis. The vision loss was complete, and pupils were dilated bilaterally without reaction to light.
A whole body CT scan in search of deep parasitic disease or infections revealed bilateral pulmonary infiltrations. Cerebrospinal fluid cytology and chemistry were normal including protein immunoelectrophoresis. Elevated blood creatine kinase levels (2900 UI/litre) suggested rhabdomyolysis, which was thought to explain the motor deficit. A complete screening for tropical parasites and bacterial or viral infections was negative. Echocardiography was normal. Electroneuromyography data, obtained 10 days after disappearance of muscle weakness under corticosteroid treatment disclosed a left median nerve axonal neuropathy by showing a reduction of 50% for muscle action potentials voltages in abductor pollicis brevis and of 68% for sensory fibres action potentials voltages in the left median nerve, as compared to values measured in the right median nerve, with no proximal or distal motor conduction slowing and no abnormality of muscle action potentials outside the left median nerve territory. A nasal mucosa biopsy showed an inflammatory mucosa with non-specific polymorphic infiltrates. An early MRI of the brain was normal, including sequences focused on the optic nerves, aside from a bilateral frontal sinusitis. Spinal MRI was also normal. Serum antibodies to neutrophilic cytoplasmic antigens were present with an elevated titre of anti-myeloperoxydase antibodies (181, normal <20) and an increased level of IgE (238 KU/litre, normal <150).
Initial ophthalmological findings before the occurrence of blindness were paleness of the optic discs and arterial thinning of the retina with arteriovenous crossing signs. At 1 week after onset of blindness, ophthalmological examination was suggestive of acute bilateral ischaemic anterior ON. His retinal vessels looked normal and, apart from paleness of the optic discs, the only abnormality was a minor retinal haemorrhage in the right eye. Fluorescein angiography showed preserved retinal vessels (figure 1). Pattern ERGs and VEPs could not be obtained because the patient was unable to maintain fixation on the centre of the screen. Flash ERGs showed normal retinal responses. White flash VEPs showed low amplitude peaks III and IV that were reproducible on three consecutive runs of right eye stimulations. Virtually no cortical response was obtained to left eye stimulation (figure 2).
Figure 1
Figure 1
Fluorescein angiography of the right eye (left) and left eye (right) before injection (upper left), and 1 min (upper right), 3 min (lower left) and 6 min (lower right) after injection of fluorescein. Retinal vessels are of normal calibre and aspect. A (more ...)
Figure 2
Figure 2
Flash visual evoked potentials (VEPs) and electroretinogram (ERG): ERG responses recorded with an active electrode placed on the inferior eyelid of the stimulated eye and bilateral earlobe reference electrodes are shown in the top and bottom traces. VEPs (more ...)
Treatment
Faced with this highly probable CSS vasculitis, we treated the patient with 1 g/day of methylprednisolone on admission in our department, 2 days after onset of blindness. The effect was initially spectacular on fever, myalgia and muscle weakness, blood eosinophilic count and systemic inflammatory response, but vision did not improve. This treatment was subsequently changed to cyclophosphamide and oral prednisone (1 mg/kg) with prophylactic antibiotherapy (ivermectine and trimethoprim sulfamethoxazole).
Outcome and follow-up
At 2.5 months after blindness onset, exposure to bright light evoked phosphenes, but without useful vision for daily activities. No visual improvement has occurred over a follow-up period of 18 months.
This case illustrates the fact that acute neurological and neuro-ophthalmological symptoms can be the first manifestations of CSS. Muscle involvement mimicking myositis is rare.11 The transient muscle weakness and pains that were reversible in 24 h under corticosteroid treatment in our patient and associated to elevated blood creatine kinase were considered likely to reflect such a process. However this interpretation does not fully explain the right predominance of the motor deficit in upper and lower limbs, for which brain and spinal MRI provided no further explanation. There were no clinical signs of rhabdomyolysis (no dark urines) and no factors predisposing to rhabdomyolysis. Because of the complete reversibility of muscle symptoms no further investigations were undertaken, in particular muscle biopsy was not carried out.
Sudden blindness due to bilateral ischaemic ON can be dramatic. Only a few cases of ischaemic ON in this context have been reported in the literature410 and are reviewed in table 1.
Table 1
Table 1
Published cases of ischaemic optic neuropathy in Churg–Strauss syndrome (CSS)
Ischaemic ON usually occurs early in the course of the disease, as in our patient; in a few case reports it was the first manifestation of the disease.410 However, simultaneous bilateral onset is exceptional4; most often the eyes are successively affected over a time course of a few weeks or a few months.6 7 Sometimes ON can be asymptomatic and discovered fortuitously.5
To date only a few cases documented by electrophysiological investigations have been published. In one 61-year-old man with bilateral but not simultaneous ischaemic ON, flash ERG was performed showing preserved a and b waves but VEPs were not recorded.6 In our patient, the association of preserved ERGs and markedly abnormal VEPs, together with the eye fundus examination and fluorescein angiography results, is highly suggestive of retrobulbar ON caused by acute anterior optic nerve ischaemia.
The prognosis for vision recovery after anterior ischaemic ON in CSS is usually poor with no or little visual recovery.410 Our patient also had poor visual outcome. The delay of 72 h between visual loss and intravenous corticosteroid treatment occurred due to the initial diagnostic difficulties.
Our case as well as the few others reported in the literature strongly suggests that the occurrence of visual loss in the context of systemic inflammation with hypereosinophilia should lead to considering the diagnosis of CSS and to prompt corticosteroid treatment, even if there is no certitude whether prompt treatment would help to achieve functional recovery.
Learning points
  • [triangle]
    Churg–Strauss syndrome (CSS) is a rare cause of sudden blindness.
  • [triangle]
    Electrophysiological explorations are useful to identify the precise mechanism of blindness.
  • [triangle]
    Visual loss in the context of systemic inflammation should lead to considering the diagnosis of CSS and to prompt corticosteroid treatment.
Acknowledgments
We are grateful to Drs Alexander Hammers and Rolf Heckemann for their comprehensive review, and English improvement.
Footnotes
Competing interests None.
Patient consent Obtained.
1. Churg J, Strauss L. Allergic granulomatosis, allergic angiitis, and periarteritis nodosa. Am J Pathol 1951;27:277–301. [PubMed]
2. Noth I, Strek ME, Leff AR. Churg-Strauss syndrome. Lancet 2003;361:587–94. [PubMed]
3. Takanashi T, Uchida S, Arita M, et al. Orbital inflammatory pseudotumor and ischemic vasculitis in Churg-Strauss syndrome: report of two cases and review of the literature. Ophthalmology 2001;108:1129–33. [PubMed]
4. Partal A, Moshfeghi DM, Alcorn D. Churg-Strauss syndrome in a child: retina and optic nerve findings. Br J Ophthalmol 2004;88:971–2. [PMC free article] [PubMed]
5. Vitali C, Genovesi-Ebert F, Romani A, et al. Ophthalmological and neuro-ophthalmological involvement in Churg-Strauss syndrome: a case report. Graefes Arch Clin Exp Ophthalmol 1996;234:404–8. [PubMed]
6. Weinstein JM, Chui H, Lane S, et al. Churg-Strauss syndrome (allergic granulomatous angiitis). Neuro-ophthalmologic manifestations. Arch Ophthalmol 1983;101:1217–20. [PubMed]
7. Acheson JF, Cockerell OC, Bentley CR, et al. Churg-Strauss vasculitis presenting with severe visual loss due to bilateral sequential optic neuropathy. Br J Ophthalmol 1993;77:118–19. [PMC free article] [PubMed]
8. Alberts AR, Lasonde R, Ackerman KR, et al. Reversible monocular blindness complicating Churg-Strauss syndrome. J Rheumatol 1994;21:363–5. [PubMed]
9. Giorgi D, Laganü B, Giorgi A, et al. Ischemic optic neuritis in Churg-Strauss syndrome. Recenti Prog Med 1997;88:273–5. [PubMed]
10. Kattah JC, Chrousos GA, Katz PA, et al. Anterior ischemic optic neuropathy in Churg-Strauss syndrome. Neurology 1994;44:2200–2. [PubMed]
11. De Vlam K, De Keyser F, Goemaere S, et al. Churg-Strauss syndrome presenting as polymyositis. Clin Exp Rheumatol 1995;13:505–7. [PubMed]
12. Hayakawa K, Akatsuka I, Matsukura S, et al. Case of anterior ischemic optic neuropathy accompanied by Churg-Strauss syndrome (article in Japanese). Nippon Ganka Gakkai Zasshi. 2004;108:612–7. [PubMed]
Articles from BMJ Case Reports are provided here courtesy of
BMJ Group