Horner’s syndrome with an ipsilateral X nerve palsy following presumed shingles

Abstract

Herpes zoster is a common clinical problem but its complications, apart from post-herpetic neuralgia, are comparatively rare. We describe a case of Horner’s syndrome and ipsilateral vagal paresis following likely herpes zoster of the third and fourth cervical roots. This unusual combination has not, to our knowledge, been previously described.

Background

Herpes zoster may cause protean neurological complications including cranial neuropathies, radiculitis. myelitis, and encephalitis.^1–4 We describe a case of Horner’s syndrome and ipsilateral vagal paresis following likely herpes zoster of the third and fourth cervical roots.

Case presentation

A previously healthy 64-year-old right handed lorry driver presented in July 2007 with a 2 day history of sudden onset dysphagia and dysarthria, 3 weeks after developing a prickling vesicular rash over the right posterior aspect of his neck with subsequent right facial pain.

The dysphagia was unassociated with odynophagia or difficulty chewing. There was no dysgeusia, visual disturbance, nor problems with limb, autonomic or sphincter function.

A longstanding unchanging asymmetrical hearing loss attributed to otosclerosis was noted. Examination revealed healing lesions characteristic of herpetic vesicles over the right C3 and C4 dermatomes. A nasal, non-fatigable dysarthria was apparent. A right Horner’s was present (fig 1) with associated palatal weakness (fig 2) and bovine cough.

Figure 1.

Right Horner’s syndrome.

Figure 2.

Palatal paralysis.

Cranial nerves IX, XI, and XII were intact. Fibreoptic nasendoscopy revealed reduced excursion of the right vocal cord with pooling of saliva. The general medical examination was otherwise non-contributory.

A clinical diagnosis of herpes zoster associated cranial polyradiculoneuritis was made and antiviral therapy with corticosteroids were commenced. Feeding continued via a nasogastric tube.

Recovery followed and the patient was discharged home 8 days later tube-free, asymptomatic, and taking a normal diet.

Investigations

A lymphopenia (0.9×10⁹/l (normal range 1.5–4.0)) was found but haematology and biochemistry, chest and cervical spine plain radiography were unremarkable.

Computed tomography and subsequent magnetic resonance imaging of the brain with gadolinium demonstrated appearances consistent with mild non-specific ischaemia with no evidence of carotid dissection or pathological enhancement.

Cerebrospinal fluid (CSF) analysis was unremarkable with negative varicella zoster (VZ), herpes simplex virus (HSV) and enterovirus PCR and cytology.

Treatment

• Dexamethasone and intravenous aciclovir.

Discussion

Horner’s syndrome is described in association with ophthalmic^5,6 or thoracic zoster^7–9 causing both peripheral and central disruption of oculosympathetic function, respectively. In addition vagal mononeuritis of viral aetiology, including herpetic, has been reported.^10–15

Pupillary reactions to hydroxyamphetamine can differentiate between a peripheral (post-superior cervical ganglionic) from central (pre- (superior cervical) ganglionic) lesion with pupillary dilatation in the latter but not the former. Although this was not undertaken in our case, the occurrence of facial pain may implicate the sympathetic fibres in the carotid sheath producing a painful Horner’s syndrome also seen in carotid artery dissection.

Furthermore, the coexistence of an isolated ipsilateral X palsy without other cranial nerve involvement or long tract signs is consistent with a multifocal peripherally sited inflammatory process rather than a central disturbance.

The transient nature of the Horner’s syndrome in this patient with resolution in 12 weeks has been described in a post-ganglionic lesion¹⁶ in contrast to its persistence (years) being reported in pre-ganglionic lesions.⁸

The lack of corroborative PCR studies in CSF is surprising and the herpetic aetiology of this illness cannot be confirmed with certainty. However, the diverse clinical complications of varicella zoster can be mediated by numerous pathological mechanisms including immunological and host inflammatory granulomatous reactions to infection,¹⁷ and in most clinical situations this diagnosis is established by the characteristic rash.

Learning points

• Awareness of the diverse complications of herpes zoster including cranial neuropathies.

• Complications can occur after the rash has disappeared, suggesting possible post-infectious/para-infectious mechanisms.

• Early treatment with antivirals and corticosteroids may aid recovery.