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Trigeminal autonomic cephalalgia-tic-like syndrome associated with a pontine tumour in a one year old girl
  1. J A van Vliet1,
  2. M D Ferrari1,
  3. J Haan1,
  4. L A E M Laan1,
  5. JHC Voormolen2
  1. 1Department of Neurology, Leiden University Medical Centre, PO Box 9600, 2300 RC Leiden, Netherlands
  2. 2Department of Neurosurgery, Leiden University Medical Centre
  1. Correspondence to: Dr Laan;
 l.a.e.m.laan.neurology{at}lumc.nl

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The so called trigeminal autonomic cephalalgias (TAC) include episodic and chronic paroxysmal hemicrania (CPH), short lasting unilateral neuralgiform headache with conjunctival injection and tearing (SUNCT), and cluster headache (CH).1 Combinations of cluster headache and chronic paroxysmal hemicrania with trigeminal neuralgia have also been described and have been called cluster-tic syndrome2 or CPH-tic syndrome.3 In order to diagnose TACs, it is essential to record the case history carefully. Only rarely have intracranial lesions such as aneurysms or tumours been observed in association with TACs. In the majority of cases, no brain abnormalities are found using conventional imaging.

We describe a three year old girl who suffered attacks of severe right sided temporal pain and autonomic disturbances and in addition neuralgic shooting pains associated with a pilocytic astrocytoma in the pons and medulla oblongata, extending to the upper cervical cord. The attacks disappeared once the tumour had been debulked.

Case report

A three year old girl presented with a history of extremely painful right sided temporal headache attacks since the age of one year. During these attacks she would grab her right ear and cry intensely. Her eyelids were slightly swollen, with rhinorrhoea on the right side. The attacks usually lasted 12 to 24 hours and occurred weekly, sometimes waking her up at night. During the attacks, the patient wanted to be in a darkened room, but she did not feel nauseous and did not vomit. Initially, neurological examination revealed no abnormalities. Aspirin or other simple analgesics were not effective. There was no family history of migraine or other severe headaches.

A video recording of several attacks made by her parents showed the child lying still while crying intensely. Her right upper and lower eyelids were swollen and tear production and rhinorrhoea were seen on the right side. It seemed that she was in continuous pain, with superimposed paroxysms of very intense pain, lasting seconds, during which she rocked back and forth (the video recording is available at the JNNP website: www.jnnp.com). These attacks occurred spontaneously at any time of day or night with no particular regularity or trigger points. Magnetic resonance imaging (MRI) of the brain revealed a tumour in the pons, extending to the medulla oblongata and cervical myelum (fig 1A), with a syrinx in the cervical myelum. On the transverse slide, the tumour extended to the cerebropontine cistern on the right side (fig 1B). Repeated physical examinations after six weeks showed hyperreflexia of the right arm and leg and positive Babinski reflexes on both sides. After debulking the tumour, the attacks resolved completely and neurological examination normalised. Pathological examination of the tumour revealed a pilocytic astrocytoma. The patient has remained headache-free following neurosurgery during two years of follow up. Neurological examination has remained normal during this period.

Figure 1

(A) T1 weighted sagittal magnetic resonance image (MRI) scan of the brain after gadolinium contrast, showing a space occupying lesion in the pons, extending to the medulla oblongata. Below the tumour a syrinx is present in the cervical myelum. (B) T2 weighted transverse MRI scan, showing extension of the tumour to the right cerebropontine angle.

Comment

The diagnosis of primary headaches associated with autonomic symptoms, such as cluster headache or chronic paroxysmal hemicrania, is based on the patients’ history, because diagnostic tests are not available. As shown here, a video recording of the attacks may be very helpful when patients are unable to describe the attacks in detail themselves. In this case the autonomic symptoms during the headache and the sudden additional shooting pains were recognised by the parents and the physician after studying these video recordings. Although the attacks lasted 12 to 24 hours, the combined headaches best resembled a combination of a TAC-like syndrome in association with trigeminal neuralgia or idiopathic stabbing headache (ISH). It is difficult to distinguish trigeminal neuralgia from ISH in this case, as both headaches only last seconds, may occur many times per day, and may be located in the first division of the trigeminal nerve. ISH is often described in association with other primary headaches and usually responds to indomethacin, in contrast to trigeminal neuralgia.1 We cannot conclude with certainty that the stabs in this case are ISH, because indomethacin was not used.

The attacks were found to be associated with a pilocytic astrocytoma in the pons that extended to the medulla oblongata on the same side as the attacks. Similar cases have suggested involvement of vascular or neoplastic intracranial lesions in TACs.1,4,5 A causal relation between the symptoms and the lesions was not easily established in all cases, but involvement of the intracranial lesions with trigeminal structures was suggested. One patient with an upper cervical meningioma had cluster headache attacks, possibly caused by direct compression of the C1–C3 rootlets or the trigeminal nucleus.6 Furthermore, cluster-tic syndrome has been reported in association with intracranial pathology.2 In this case, direct compression of the trigeminal root by the basilar artery entering deep into the cerebellopontine cistern was suggested as a cause of the pain. It is not clear how the lesion in our case caused paroxysmal symptoms, but disappearance of the symptoms after debulking suggests a causal relation.

References

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    Trigeminal autonomic cephalalgia-tic-like syndrome associated with a pontine tumour in a one year old girl
    J A van Vliet, M D Ferrari, J Haan, L A E M Laan, and JHC Voormolen


    Web-only Video

    This video recording, made by the parents of the girl in the case report, shows several attacks, with the child lying still while crying intensely. 
    Her right upper and lower eyelids are swollen and tear production and rhinorrhoea are seen on the right side. It seems that she is in continuous pain, with superimposed paroxysms of very intense pain, lasting seconds, during which she rocks back and forth.
    [View Video]

Footnotes

  • Competing interests: none declared

  • A video recording of the patient during a headache attack is available on the journal website (www.jnnp.com). The recording shows the patient sitting still with a swollen eyelid on the right side. A few moments later she suddenly starts to cry intensely, while grabbing to her right eye and right ear. During this episode, she moves back and forth, and pronounced lacrymation is seen in her right eye.

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