Droopy eyeBMJ 2006; 332 doi: https://doi.org/10.1136/sbmj.0605194 (Published 01 May 2006) Cite this as: BMJ 2006;332:0605194
- Akheel A Syed, specialist registrar1,
- R Deeba Syeda, preregistration house officer2,
- Farheena N Mecci, clinical tutor3
A woman aged 83 presented with increasing shortness of breath of a few weeks' duration. Air entry was reduced and percussion on the right side of her chest produced a dull note. The skin on the right side of her face was dry, in addition to the features seen in the photograph (fig 1).
Fig 1 Note the drooping of the upper eyelid and the smaller pupil on the right; the right side of her face was also dry to the touch.
What neurological diagnosis is suggested by the findings in her face?
What investigation will you perform next? What is the underlying diagnosis?
She had right sided ptosis, miosis, and anhidrosis, features that characterise an oculosympathetic palsy known as Horner's syndrome.
A postero-anterior chest x ray was performed (fig 2, panel A). This showed opacification of the right upper zone with pleural effusion of a moderate volume; a computed tomography scan confirmed a primary bronchogenic carcinoma in the posterior part of the right upper lobe and right sided pleural effusion (fig 2, panel B), and metastatic tumour nodules in the left upper, mid and lower zones as well as the right lower zone.
Johann Friedrich Horner (1831-86), a Swiss ophthalmologist, published the first full clinical description of the eponymous syndrome in a 40 year old woman, Anna Brandli, in his 1869 treatise, Uber eine Form von Ptosis [on a form of ptosis].1 However, others before him had recognised the syndrome, notably Edward Selleck Hare (1812-38), an English house surgeon, in 1838,2 and Claude Bernard (1813-78), a French experimental surgeon, in 1852. The latter performed meticulous experiments in rabbits and dogs and showed that “division of the cephalic portion of the great sympathetic” nerve was followed by miosis, ptosis, enophthalmos, relaxation of the nictitating membrane, reduction of intraocular tension, decrease in the size of the nares, and an increase in temperature.34 Thus, oculosympathetic palsy is sometimes also referred to as a Claude Bernard syndrome or Bernard-Horner syndrome.
Horner's syndrome is an uncommon condition with no known predilection for race, sex, or age. It can occur as a result of a lesion anywhere along the oculosympathetic pathway, which includes the central (first order) neurons in the brain, the preganglionic (second order) neurons in the cervical sympathetic outflow, and the postganglionic (third order) neurons that supply the levator palpebrae superioris (Muller muscle), the dilator pupillae, and the sweat glands of the face (fig 3).
Pharmacological tests based on the ability of the normal (unimpaired) sympathetic postganglionic nerve to synthesise and release norepinephrine at its terminals may be used to localise the level of the lesion. 5 Instillation of a 10% solution of cocaine in the conjunctival sac causes mydriasis in the normal eye by inhibiting reuptake of synaptic norepinephrine. There is no dilatation of the pupil in the affected eye even after 45 minutes, confirming the presence of the lesion in the preganglionic or postganglionic neurons because the affected neurons will fail to elaborate norepinephrine at the terminals. If the lesion is central, there will be a slight dilatation, although much less than on the normal side. 5 A 1% solution of paredrine (hydroxyamphetamine hydrobromide) enhances the release of norepinephrine from sympathetic terminals and causes mydriasis.5 As intact postganglionic neurons have the potential to produce norepinephrine, a normal response to paredrine in a patient with Horner's syndrome implies that the lesion is in the central or preganglionic neurons. No dilatation will occur in the presence of a postganglionic lesion due to degeneration of terminals.
Association of carcinoma of the apex of the lung or Pancoast's tumour (Henry Khunrath Pancoast (1875-1939), American radiologist) 6 with destructive lesions of the thoracic inlet and involvement of the brachial plexus (resulting in wasting of hand muscles) and cervical sympathetic nerves (resulting in ipsilateral Horner's syndrome) is known as Pancoast's syndrome.67 Although Pancoast's tumour is the most important cause of Horner's syndrome, 6 it can result from numerous other lesions as well (box 1) 8 and treatment, where possible, should be directed at the underlying cause.
Causes of Horner's syndrome
Central lesions (first order neuron) Arnold-Chiari malformation
Basal meningitis (for example, syphilis)
Base of skull tumours
Cerebrovascular accidents or Wallenberg syndrome
Demyelinating disease (such as multiple sclerosis)
Preganglionic lesions (second order neuron)
Aneurysm or dissection of aorta, subclavian artery, or common carotid artery
Central venous catheterisation
Chest tube insertion
Lymphadenopathy (such as Hodgkin's disease, tuberculosis, leukaemia, mediastinal tumours)
Surgical injury (such as thyroidectomy, radical neck dissection, carotid angiography)
Middle ear lesions (such as acute otitis media)
Mandibular tooth abscess
Birth trauma with injury to lower brachial plexus
Postganglionic lesions (third order neuron)
Internal carotid artery dissection
Carotid cavernous fistula
Cluster or migraine headaches
Raeder syndrome (paratrigeminal syndrome)
Drugs (may affect any region)
Local anaesthetic agents (such as lidocaine, bupivacaine)
Antipsychotics (such as thioridazine, trifluoperazine)
Opiates (such as diamorphine)
Anti-parkinsonism agents (such as levodopa)
Anti-hypertensive agents (such as guanethidine, reserpine)
Influenza virus vaccine
Originally published as: Student BMJ 2006;14:194