CASO CLÍNICO

Trigeminal neuralgia secondary to cholesterol granuloma of the petrous bone apex

Neuropatía sensitiva trigeminal secundaria a granuloma de colesterol de la punta del peñasco del temporal

M.A. Pons García¹, C. Moreno García²

1 Neuróloga. Servicio de Neurología.
2 Cirujano Oral y Maxilofacial.
Servicio de Cirugía Oral y Maxilofacial. Hospital Infanta Cristina. Badajoz. España

 Correspondence

ABSTRACT

Trigeminal Neuralgia is an uncommon entity. The patients report hypoesthesia and/or dysesthesia of the second and third ramus of trigeminal nerve, while neuralgia is very rare.¹ Its association with systemic diseases of connective tissue is well know.² It has been described as being associated with different lesions of the central nervous system, especially with the posterior cavity or cranial base tumors, as well as jaw neoplasias.^3,4 We presented a patient with hypoesthesia V2-V3 and hemi facial paroxysmal pain secondary to lesion of petrous apex of temporal bone.

Key words: Trigeminal neuropathy; Orofacial hypoalgesia; Petrous apex of temporal bone.

RESUMEN

La neuropatía aislada de la rama sensitiva del trigémino es una entidad poco habitual. Los pacientes suelen referir hipoestesia y /o disestesia generalmente a nivel de la segunda y tercera rama del trigémino, mientras que la neuralgia es muy infrecuente.¹ Su asociación con enfermedades sistémicas del tejido conectivo es bien conocida.² Se ha descrito asociada a distintas lesiones del SNC sobre todo tumores de fosa posterior o base de cráneo, así como neoplasias mandibulares.^3,4 Presentamos una paciente con hipoestesia en el territorio V2-V3 asociada a dolor hemifacial paroxístico secundario a una lesión del peñasco del temporal.

Palabras clave: Neuropatía trigeminal; Hipoestesia orofacial; Peñasco del temporal.

Introduction

Trigeminal neuralgia involves a hypoesthesia and/or dysesthesia sensation and it appears either suddenly or gradually. It is generally limited to the second or third branch of the trigeminal nerve, lasts for a variable amount of time and does not have an associated motor deficit. It is an uncommon pathology, although it is well known by experts in the field and in the last few years it has been a topic of interest. It has been described as being associated with connective tissue disease, central nervous system infections, vascular enlargement and different types of tumors, especially meningiomas. The most common site for these lesions is the posterior cavity.^5,6 According to the literature reviewed, its association with fatty apex tumors of the petrous bone is exceptional, that is what sparked our interest and made us decide to present this case.

Clinical Case

60 year-old female with a medical history of dyslipidemia and chronic tension cephalea. She goes to the doctor because of pain episodes in the left side of the face that last a few seconds and have been going on for the last 2 months. The pain is shooting/stabbing and takes place in the second and third branch of the trigeminal nerve.

The episodes are provoked by the friction in the face when laughing or chewing and afterwards there is a sensation of hypoesthesia and swelling in this site. It can happen up to three times a day. The patient does not report cephalea, red eye, weeping or runny nose. There are no abnormal movements in this side of the face, dysphagia or other symptoms.

The physical exam reports the patient as vigil, oriented and cooperative. There is isochoric and pupil normorreaccion, eye movement is normal and so is the back of the eye. Examination of the cranial pair is normal except that a hypoesthesia is in the 2nd and 3rd left branch. There is no dysphagia or changes in speech. There is no motor deficit or associated sensitivity in other areas. Reflexes are present and symmetric. The rest of the neurological and general exam is normal. They ask for analysis to rule out hypercholesterolemia and hypertriglyceridmia. The brain MRI (Figs. 1 and 2) increased the power in T1, T2 and FLAIR with fat suppressing sequences that caused the lengthening the diameter of the left petrous apex 28 mm. It touches the posterior sphenoid sinus and small ethmoidal cells like the intrapetrous carotid. This takes place without changing its flow that doesn't enhance after administering the contrast; it is compatible with cholesterol granuloma of the point of the petrous bone. We also casually discovered an empty sella turcica.

After evaluating the patient we establish 400 mg/day of carbamazepine is appropriate to treat the symptoms. 48 hours after starting treatment the patient has no symptoms, both the pain and hypoesthesia have disappeared, opting for conservative treatment at the moment.

Discussion

Trigeminal neuralgias that originate from tumors make up a small percentage of trigeminal neuralgias. They start as cutaneous-mucosa drowsiness in the region of the trigeminal, mainly affecting the V2 and V3, especially in the maxilla branch. Its importance is established by its ability to be the first clinical manifestation (benign or malignant) or relapse in patients with previous neoplasias. Of these, meningioma of the posterior cavity is the most frequent type and location.

However, the most described association was of systematic illnesses, particularly of systematic sclerosis, mixed conjunctive tissue disease and Sjorgen syndrome. The difference is that in this case there aren't usually manifestations prior to the disease. Sensitive neuropathy trigemina has been described in relation to infections (herpes zoster, neurosyphilis, and tuberculosis) arachnoiditis and traumas.^8,9

The trigeminal nerve can be damaged in different areas from the encephalon trunk, preganglionated roots and Gasser ganglion to the portion of the peripheral nerve. The Gasser ganglion is located in the upper anterior portion of the apex of the temporal bone.

The most frequent clinic is hypoesthesia and/or dysesthesia and the least frequent is neuralgia. The majority of patients describe dysesthesia as being moderately intense, 60% report occasional neuropathic pain and 80% have hypoesthesia upon neurological exploration.⁸ The malignant mental neuropathy is characterized by spontaneous anesthesia of the region of the mental nerve, and the sensitive deficit that includes the chin skin and the inferior lip mucosa. Among those that have cholesterol granulomas, fatty tumors are very uncommon. They are 0.1% of all the intracranial tumors and are generally asymptomatic. They are normally found in the mediosagittal, close to the callous body and cerebella point. They are sometimes found, but not very often, in the dorsal mesencephalon, vermiform lobe, medulla and spinal cord base.^10-12

It is absolutely necessary to perform a complete radiological study of the trigeminal neuralgia in order to rule out brain and trunk lesions as well as preganglionated roots or Gasser ganglion and cavernous sinus. Just like in the extra cranial zone all along the 3 branches of the V cranial nerve.¹²

Brain image techniques help us carry out the differential diagnostic of the distinct lesions that affect the petrous bone point. The cholesterol granuloma is shown in an expansive process, hyper intense in the CAT where there is also adjacent bone erosion and the hyper intense MRI with a potency of T1, T2, Flair and proton density, cancelling its signal in fat suppressing sequences.^12,13 The central cavity is a-vascular, doesn't enhance the contrast and we are able to see areas with high hypo signal through the granulated tissue. For example areas of hyper signal, because of its high protein content and repetitive bleeding. The muccocell is uncommon and its MRI is variable. If it is recent it is usually hypo dense in the T1 because of its low protein content and if it's chronic it is shiny in T1 and T2. Its mucosa wall is enhanced by the contrast. Another entity is the first cholesteatoma or episidermoide of the point petrous bone that looks like an expansive lesion that is isodense or hypo dense in the CT, hypo dense in the T1 and hyper intense in T2 of the MRI and whose wall is reinforced with contrast. In any case the definitive diagnostic is the anantomicopathological study.¹⁴

Regarding treatment, once the illness has been diagnosed symptomatic treatment should be started. Carbamazepine is the treatment of choice although other pharmaceuticals have been used in neuralgias. In the case of progressive or uncontrollable symptoms of benign tumors or in the case of malignant neoplasias the treatment should be surgical. The treatment approach chosen for a cholesterol granuloma at the petrous bone point was preauricular infra temporal.^15,16

In our case, because it is a benign tumor it presents an excellent response to carbamazepine symptomatic treatment, we choose a conservative attitude at the moment.

Correspondence:
Carlos Moreno García
Servicio Cirugía y Maxilofacial
Hospital Infanta Cristina.
Crta. de Portugal, s/n. Badajoz
E-mail: carlosmorenogar@gmail.com

Recibido: 23.11.2008
Aceptado: 24.09.2009

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