Sir,
The headache, neurologic deficits, and cerebrospinal fluid (CSF) lymphocytosis (HaNDL) syndrome is characterized by a temporary neurological deficit followed by a severe headache with persistent CSF lymphocytosis. Other features include an increased CSF protein, an increased CSF opening pressure, and a viral prodrome. The exact pathogenesis of HaNDL is unknown.1 We report a patient with HaNDL syndrome presenting with complete external ophthalmoplegia.
Case report
A healthy 18-year-old woman presented with a 1-week history of headache, nausea, vomiting, and fever. She developed severe visual loss over 4 days followed by acute ophthalmoplegia.
Her best-corrected visual acuity was hand motions, OD, and light perception OS. Humphrey visual fields revealed severe peripheral constriction OU. Her pupils were round and 6 mm; she had no relative afferent pupillary defect. She had bilateral ptosis and no extraocular motility. Vestibulo-ocular reflexes were absent. Anterior chamber examination was normal. Optic discs were swollen with slit haemorrhages in the perimacular area. She had diminished light touch in patchy areas over her upper extremities.
Magnetic resonance imaging (MRI) of the brain and MR venogram of the brain with and without contrast were normal. CSF revealed an elevated opening pressure of 320 mm H20, 172 lymphocytes, 6 red blood cells, protein of 92 mg per 100 ml, and glucose of 55 mg per 100 ml. CSF VDRL and Lyme titres were negative. CSF cultures and PCR for HIV, HSV 1 and 2, and EBV were all negative. CSF IgG synthesis rate and oligoclonal bands were negative. TSH, antinuclear antibody, and acetylcholine receptor antibodies were normal. Single-fibre electromyography was unremarkable.
After CSF drainage with repeated lumbar punctures and receiving i.v. methylprednisolone 1 g/day for 5 days, her symptoms and signs resolved in several weeks, and her final visual acuity was 20/20 OU.
Comment
Blurred vision, photophobia, homonymous hemianopsia, photopsias, cortical blindness, papilledema, and sixth nerve palsies have previously been associated with HaNDL.2 Although HaNDL is recognized as a distinct entity (IHS 7.8) in the International Headache Society Classification ICHD-II,3 monophasic cases like this could also represent unusual forms of viral meningitis. In addition to the self-limiting features of headache, dysesthesias, CSF lymphocytosis, and papilledema, this patient presents with transient complete external ophthalmoplegia, a neuro-ophthalmic sign not previously observed in HaNDL. External ophthalmoplegia can also be seen in idiopathic intracranial hypertension (IIH) and usually resolves with lowering of intracranial pressure.4 Ophthalmoplegia in HaNDL may arise from the effects of increased intracranial pressure and resolve with the lowering of CSF pressure in a similar manner to IIH.
Therefore, transient ophthalmoplegia should be recognized as part of the clinical spectrum of HaNDL syndrome. Although it is an uncommon disorder, ophthalmologists should include HaNDL in the differential diagnosis of any patient with papilloedema and transient neuro-ophthalmic signs.
References
Berg MJ, Williams LS . The transient syndrome of headache with neurologic deficits and CSF lymphocytosis. Neurology 1995; 45: 1648–1654.
Morrison DG, Phuah HK, Reddy AT, Dure LS, Kline LB . Ophthalmologic involvement in the syndrome of headache, neurologic deficits, and cerebrospinal fluid lymphocytosis. Ophthalmology 2003; 110 (1): 115–118.
International Headache Society. IHS Classification ICHD-II. http://www.ihs-classification.org/en/ (accessed on 23 December 2008).
Bruce BB, Newman NJ, Biousse V . Ophthalmoparesis in idiopathic intracranial hypertension. Am J Ophthalmol 2006; 142 (5): 878–880.
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Chan, J., Cheng, C. Complete external ophthalmoplegia in headache, neurologic deficits, and cerebrospinal fluid lymphocytosis (HaNDL) syndrome. Eye 24, 198–199 (2010). https://doi.org/10.1038/eye.2009.31
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DOI: https://doi.org/10.1038/eye.2009.31
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