Cluster headache
Short Summary

A 40 years old female has been suffering from cluster headache for the last 20 years. For the last 1.5 years it has been transformed from an episodic form to a chronic one. The patient reports an episodes of vertigo, for which she carried out otorhinolaryngology examination and brain NMR. On NMR was detected a periventricular lesion of about 1.2 cm, without mass effect. This lesion was probably present also 4 years ago, when the patient underwent another NMR. Follow up NMR was done month later with contrast medium and angio-MR- the lesion has not changed and angio-MR has not revealed pathological findings. Following these medical tests, the patient underwent a neurological examination,  that have excluded a demyelinating disease. The neurologist has expressed, therefore, the opinion that the encephalic lesion at issue is to be interpreted as a tumoral lesion even though of non-aggressive nature.

The patient was sent to neurosurgery hospital where the doctors, who assessed her conditions, were very evasive and did not give any importance to the encephalic lesion detected by the NMR. They only confirmed a follow-up after 6 months since they believe that, as the two NMRs were carried out with two different machineries, they are not comparable.

In the meantime, the neurologist prescribed Laroxyl 40 mg (5 drops in the evening) and Synflex 550 mg when needed (the patient informs us that for 20 years she has taken many drugs to heal her cluster headache) without any relief until now.

Patient's Questions

1) What do you think is the most probable diagnosis regarding the encephalic lesion? Further necessary tests to carry out?
2) Do you think that this lesion is connected to the cluster headache and its increased intensity?
3) What therapy do you suggest?
4) Prognosis?
 

Medical Background

40 years old, female.

For about twenty years the patient has suffered from cluster headache, that in the last year and a half has become chronic, occurring every day without pauses also when the acute phase of the cluster ends. The patient reports, an episode (it was not the first one occurred) of vertigo, for which she carried out otorhinolaryngology examination in the emergency room.

In otorhinolaryngologist summary: appearance starting from the morning hours of subjective vertigo, with nausea, no vomiting, no hearing disorder in patient with history of cluster headache. At the objective examination: no spontaneous and positional nystagmus, no deviations, no dysmetria; she cannot carry out the Romberg’s test: no ataxy. In conclusion of otorhinolaryngologist the picture is not clear and he advised symptomatic therapy (antiemetics) and neurological consultation: if this were negative the patient can be discharged and examined in the following days if an improvement does not occur.

The ptient carried out MR of the brain from which a not well defined lesion was detected with the following medical report:
“Altered signal area is appreciated, with hyper-intensity on the long TR sequences and on FLAIR images, ovalar shaped, of about 1.2 cm, localized in correspondence of the white matter of the right parietal radiate crown, near the cella media of the right lateral ventricle, without any mass effect, that is advisable to be investigated with contrast medium. Moreover, some punctiform signal hyper-intensity on DP-T2 and FLAIR are appreciated, also in front regions cortical-subcortical area and another one, punctiform too, at the left paramedian bulbo-medullary junction. The corpus callosum shows signal intensity within normal limits, just like the callosal-septal junctions. In correspondence of the lenticular nuclei, in particular on the right, and in the semioval centers area, presence of punctiform images, from the signal similar to liquor in all the sequences, ascribable to wider perivascular spaces. The pontine emergence of the trigeminal nerves and the acoustic-facial packages are symmetrical, of normal morphology and signal intensity bilaterally; no signs of neurovascular conflicts are appreciated, in particular on the right. The ventricular cavities show normal width and are in axis. Moderate greater width in periencephalic subarachnoid liquoral spaces, in particular in front-parietal area bilaterally.”
This lesion was probably present also 4 years ago, when the patient underwent another NMR although not indicated in the relevant medical report.

Month later NMR with contrast medium and angio-MR of the intracranial arterial circle was done for diagnostic deepening, whose medical report is reported as follows: “The altered signal area, with hyper-intensity on the long TR sequences and on FLAIR images and with hint of hypo-intensity in T1, ovalar-shaped, of about 1.2 cm, localized in correspondence of the white matter of the right parietal radiate crown, near the cella media of the right lateral ventricle and without mass effect, does not show any absorption of contrast medium: the MR picture, suggestive of demyelinating pathology, is to be defined in the neurological specialist field (instrumental and clinical-laboratory); MR follow-ups advisable to be carried out in times to come, the first one in about 3-4 months, and in any case when it is clinically advisable. No contrast medium pathological impregnations are evident in upper and lower tentorium region.
The Angio-MR sequence of the intracranial arterial circle focussed on Willis Polygon branches and on the main artery ramifications, has not revealed pathological findings, taking into consideration the resolution limits inherent to the methodology itself.”

Following these medical tests, the patient underwent a neurological examination, carrying out examinations (evoked potentials, blood tests but no CSF tests) that have excluded a demyelinating disease. The neurologist has expressed, therefore, the opinion that the encephalic lesion at issue is to be interpreted as a tumoral lesion even though of non-aggressive nature. This opinion is supported by the fact that the lesion has enlarged during the last 4 years and this would justify, according to the neurologist, the headache worsening.

The patient was sent to neurosurgery hospital where the doctors, who assessed her conditions, were very evasive and did not give any importance to the encephalic lesion detected by the NMR. They only confirmed a follow-up after 6 months since they believe that, as the two NMRs were carried out with two different machineries, they are not comparable.

In the meantime, the neurologist prescribed Laroxyl 40 mg (5 drops in the evening) and Synflex 550 mg when needed (the patient informs us that for 20 years she has taken many drugs to heal her cluster headache) without any relief until now.
 

Expert's Opinion

This patient has been suffering from cluster headache (CH) for the last 20 years. For the last 1.5 years it has been transformed from an episodic form to a chronic one.

The first question that should be raised is the association between the CH and the brain lesion demonstrated by the MRI. Almost all of the CH cases are primary only few reports demonstrate structural lesions such as large brain aneurysms and vascular malformations, pituitary macroadenomas, and brain stem lesions. Since the patient has been suffering from CH for the last 20 years and the brain lesion is hemispheric and has no mass effect I don't believe that there is any association between this lesion and the CH as well as the fact that it has been turned into a chronic form.

As for treatment, we are dealing with a chronic form of CH so there are several possibilities:
1. Medical treatment: this may include T. Verapamil 240-480 mg /day ,T.Lithium carbonate 300mg X2, T. Cafergot X 1 to be given in parallel.
    One can use Valproate, Topiramate, Melatonin or Botulinium toxin.
2. Occipital stimulation: Recently, pacing the occipital nerve bilaterally has shown good results in chronic CH and should be suggested if medical treatment fails or side effects are serious.

Since the periventricular lesion is a very slow growing tumor that has no mass effect and no focal neurological signs I suggest MRI follow up half yearly.

Chronic CH is sometimes therapy resistant and should be treated according to the clinical reaction. Steroids can be used at certain points for short periods to alleviate the burden of pain. Triptans especially Narapriptan can be used as well for prevention.

Regarding prognosis chronic CH is a chronic state and demands daily medical treatment for long periods.
 

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