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Trigeminal autonomic cephalalgias present with excruciating headaches and accompanying autonomic features such as ipsilateral lachrymation, rhinorrhoea, and eyelid ptosis. For clinical purposes, these headaches are subclassified into cluster headaches, paroxysmal hemicranias, and the SUNCT syndrome (sudden unilateral neuralgiform headache with conjunctival injection and tearing),1 of which the paroxysmal hemicrania subtypes such as the acute and chronic hemicranias are indomethacin responsive. Neuroimaging is often normal in trigeminal autonomic cephalalgias; nevertheless MRI should be considered, as there are associations between trigeminal autonomic cephalalgia and multiple sclerosis,2 lateral medullary infarction,3 pontine tumours,4 basilar aneurysms,5 and other posterior fossa lesions. We present an unusual case of recurrent transient ischaemic attacks (TIA) with symptoms reminiscent of a trigeminal autonomic cephalalgia which ceased after a ponto-mesencephalic infarct.
A 65 year old women was admitted with a three day history of stereotyped neurological episodes. These were characterised by a burning sensation in the left side of face, congestion of the left eye, lachrymation from the left eye, watery discharge from the left nostril, slurring of speech, and difficulty in walking. These episodes were not accompanied by headache. Each episode lasted for around 30 minutes and occurred three or four times a day. On examination, there were no focal neurological deficits. In hospital, she had four more episodes. As she had a left mature cataract (fig 1), the possibility of glaucoma induced by lens rupture was considered and ruled out. Computed tomography of the brain was unremarkable. The possibilities considered were a vertebro-basilar TIA and a trigeminal autonomic cephalalgia. As she had additional neurological symptoms, a TIA was considered more likely than trigeminal autonomic cephalalgia, and she was started on aspirin and intravenous heparin. Indomethacin was begun at 75 mg/day but had to be discontinued after a single dose because of gastric distress. Two days later, her episodic symptoms disappeared; however, she now complained of persistent right sided weakness and difficulty in walking. On examination, she had a new left sided Horner’s syndrome, right upper motor neurone facial palsy, and right upper limb weakness. Deep tendon reflexes were brisker on the right than on the left. Magnetic resonance imaging of the brain at this time (one week after the onset of symptoms) showed an acute infarct at the left ponto-mesencephalic junction (fig 2). At follow up 10 days later, her gait had improved. Her episodic symptoms had disappeared.
Cranio-facial autonomic symptoms such as ipsilateral lachrymation, rhinorrhoea, conjuctival congestion, ptosis, or eyelid oedema are the sine qua non of trigeminal autonomic cephalalgia. It is thought that activation of the trigeminal afferent system generates pain, and co-activation of the VIIth nerve efferent parasympathetic pathway produces the autonomic manifestations such as ipsilateral lachrymation and rhinorrhoea. A mechanism analogous to this can be evoked to explain our patient’s symptoms. She had an ischaemic stroke in the ponto-mesencephalic junction. Ischaemia in this region could involve the trigeminal main sensory and mesencephalic nuclei, resulting in facial dysaesthesia comparable to ischaemic nerve pain. In contrast to the “boring” intense pain often encountered with trigeminal autonomic cephalalgias, the burning dysaesthesias are more consistent with ischaemia. Ischaemia could also trigger trigemino-facial synapses in the superior salivatory nucleus and switch on the facial efferent parasympathetic pathway, resulting in ipsilateral lachrymation and rhinorrhoea.
To the best of our knowledge, this is the first report of a TIA presenting with trigeminal sensory-autonomic symptoms. This report highlights the expanding spectrum of trigeminal autonomic cephalalgias and emphasises the need to rule out a vertebrobasilar TIA in elderly patients with a new onset of trigeminal autonomic cephalalgia, especially if additional neurological symptoms are present.
Competing interests: none declared