Isolated hypoglossal nerve palsy: an unusual rare presentation in systemic lupus erythematosus

Paralisia isolada do nervo hipoglosso: uma apresentação rara no lupus eritematoso sistêmico

LETTERS

Isolated hypoglossal nerve palsy: an unusual rare presentation in systemic lupus erythematosus

Paralisia isolada do nervo hipoglosso: uma apresentação rara no lupus eritematoso sistêmico

Paulo José Lorenzoni^I; Rosana H. Scola^I; Cláudia S.K. Kay^I; Felipe T.M. Novak^I; Elaine H. Cardoso^I; Márcia R.R. Scalcon^II; Acir Rachid Filho^II; Lineu C. Werneck^I

^INeurology Division, Internal Medicine Department, Hospital de Clínicas, Universidade Federal do Paraná (UFPR), Curitiba PR, Brazil

^IIRheumatology Division, Internal Medicine Department, Hospital de Clínicas, Universidade Federal do Paraná (UFPR), Curitiba PR, Brazil

^{Correspondence}

Systemic lupus erythematosus (SLE) is an immune-mediated disease of unknown etiology which can damage the peripheral and central nervous system¹. Cranial nerves have rarely been involved in SLE's patients^2,3. Isolated hypoglossal nerve palsy (HNP) was even rarer reported in patient with SLE⁴.

We report a patient with SLE who presented with isolated HNP.

CASE

A 27-year-old woman presented with fever, alopecia, skin rash, photosensitivity, Raynaud's phenomenon, arthritis, joint and muscle pain associated to lymphopenia and low complement levels. Antinuclear, anti-Sm, anti-DNA and anticardiolipin (IgG) antibodies were detectable. The diagnosis of SLE was made, and after treatment with hydroxychloroquine (250 mg/day), prednisone (10 mg/day), and methotrexate (7.5 mg/week) her disease was controlled.

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At 42 years of age, she developed a sudden onset of dysarthria with minor speech difficulty that was related to tongue immobility, and poorly articulated speech for lingual consonants. She later noted that her speech was slurred and her tongue deviated to the right when protruded. She denied trauma, cervical pain, episodes of aspiration (airway protection), exposure to drugs or toxic agents.

Cranial nerve examination showed asymmetrical tongue position at rest with atrophy and weakness in right side of her tongue (Fig A). The tongue deviated to the right when protruded (Fig A). She was also unable to fully protrude the right cheek against resistance using the tongue. Gag reflex, swallowing, taste and sensation of tongue and oropharynx were normal.

The investigation yielded the following results: fibrillations and positive wave potentials were observed in needle electromyography of tongue on the right (Fig B); brain magnetic resonance imaging revealed areas of high signal in the right mastoid cells, lateral clivus region and hypoglossal nerve canal (Fig C and D); and increased reactive C protein (1.66 mg/dL; normal <0.5 mg/dL).

The SLE management with increase of prednisone (60 mg/day) controlled her disease and resulted in marked and progressive improvement of her symptoms and tongue paralyses (HNP) after few weeks.

All studies were done following informed consent.

DISCUSSION

Cranial neuropathies prevalence in SLE varies among different studies ranging between 2% and 7%². In the majority of cases, cranial neuropathies are either multiple or occur in association with deficits in the ascending and descending pathways. Isolated cranial neuropathy is very rare and only described in anecdotal case reports and small case series^3,5. This is the second case report of SLE complicated with isolated HNP⁴.

We believe that SLE predisposed our patient to develop isolated hypoglossal neuropathy, but the pathogenesis of cranial nerve palsy in SLE is unknown. In the first case reported, HNP followed fulminant acute pneumonitis in a SLE patient with kidney and pulmonary amyloidal deposit, suggesting that nerve palsy was a vascular phenomenon of SLE⁴. However, three theories have been suggested: auto-antibodies to neuronal antigens, ribosomes, and phospholipids either produced locally or having crossed an altered blood-brain barrier as well as blood-nerve barrier; vascular lesions (vasculitis or antiphospholipid antibodies mediated ischaemic/thrombotic vasculopathy); or inflammation related to local cytokine production^1,2.

Although treatment of SLE associated with cranial nerve palsy remains empirical our patient showed improvement after adjustment of prednisone dose.

Received 20 April 2011

Accepted 1 June 2011

• 1. Muscal E, Brey RB. Neurologic manifestations of systemic lupus erythematosus in children and adults. Neurol Clin 2010;28:61-73.
• 2. Bruns A, Meyer O. Neuropsychiatric manifestations of systemic lupus erythematosus. Joint Bone Spine 2006;73:639-645.
• 3. Saleh Z, Menassa J, Abbas O, Atweh S, Arayssi T. Cranial nerve VI palsy as a rare initial presentation of systemic lupus erythematosus: case report and review of the literature. Lupus 2010;19:201-205.
• 4. Chan CN, Li E, Lai FM, Pang JA. An unusual case of systemic lupus erythematosus with isolated hypoglossal nerve palsy, fulminant acute pneumonitis, and pulmonary amyloidosis. Ann Rheum Dis 1989;48:236-239.
• 5. Alves P. Imaging of hypoglossal nerve. Eur J Radiol 2010;74:368-377.

Publication Dates

• Publication in this collection
  31 Oct 2011
• Date of issue
  Oct 2011