Sir,
Acute disseminated encephalomyelitis (ADEM) is thought to have an underlying autoimmune, demyelinating aetiology. It resembles multiple sclerosis and typically occurs in children.1, 2, 3 It is characterized by diffuse neurological features including headache, cerebellar ataxia, altered mental state, choreiform movements and vertigo. The classic ophthalmic manifestation is optic neuritis. We report the first case of ADEM with optic neuritis associated with marked peri-papillary haemorrhages.
Case report
A 6-year-old boy with an 8-week history of headache and reduced vision in his left eye was referred by his neurologist. On examination his visual acuity was RE 6/24, LE 6/60, he had an RAPD in the left eye. Eye movements were full and anterior segments were quiet. Fundoscopy revealed optic disc swelling with marked peri-papillary haemorrhages with otherwise normal maculae (Figure 1a). FBC, U+E, CRP, and ESR were all normal, whereas PCR for HSV1 and 2, EBV, and CMV were all negative. LP was normal with an opening pressure of 14 mm Hg. An MR scan showed a number of peri-ventricular high-signal lesions. He was diagnosed with ADEM and optic neuritis, and treated with two doses of intravenous methyl-prednisolone followed by a reducing dose of oral prednisolone. He has made a good recovery with improved vision to 6/6 in his left eye, resolution of optic nerve swelling, and reabsorbing peri-papillary haemorrhages (Figure 1b). His right eye remains quiescent and there have been no further neurological or ophthalmic episodes.
Figure 1.
(a) Left fundus. Optic nerve swelling with peri-papillary haemorrhages. (b) Left fundus. Resolution of optic nerve swelling and reabsorbing haemorrhages.
Comment
To the best of our knowledge, this is the first report of ADEM with peri-papillary haemorrhages. This observation raises the question as to the underlying aetiology and although an autoimmune mechanism with HLA-DR linkage is likely, other forces may have a role to perform.4, 5 Further work is required to ascertain the possibility of a vasculitic element to the underlying processes.
The authors declare no conflict of interest.
References
- Lucas J. An account of uncommon symptoms succeeding the measles with additional remarks on the infection of measles and smallpox. London Med J. 1790;11:325–331. [PMC free article] [PubMed] [Google Scholar]
- Menage T, Hemmer B, Nessler S, Wiendl H, Neuhaus O, Hartung HP, et al. Acute disseminated encephalomyelitis. Arch Neurol. 2005;62 (11:1673–1680. doi: 10.1001/archneur.62.11.1673. [DOI] [PubMed] [Google Scholar]
- Murphy J, Austin J. Spontaneous infection or vaccination as cause of acute disseminated encephalomyelitis. Neuroepidemiology. 1985;4:138–145. doi: 10.1159/000110225. [DOI] [PubMed] [Google Scholar]
- Schwarz S, Mohr A, Knauth M, Wildermann B, Storch-Hagenlocher B. Acute disseminated encephalomyelitis: a follow-up study of 40 adult patients. Neurology. 2001;56:1313–1318. doi: 10.1212/wnl.56.10.1313. [DOI] [PubMed] [Google Scholar]
- Rush J. Idiopathic optic neuritis of childhood. J Pediatric Ophthalmol Strabismus. 1981;18:39–41. doi: 10.3928/0191-3913-19810501-09. [DOI] [PubMed] [Google Scholar]