Nervus intermedius and the surgical management of geniculate neuralgia.

OBJECTIVE: Geniculate neuralgia (GN) is an uncommon craniofacial pain syndrome attributable to nervus intermedius (NI) dysfunction. Diagnosis and treatment can be challenging, due to the complex nature of ear sensory innervation, resulting in clinical overlap with trigeminal neuralgia (TN) and glossopharyngeal neuralgia (GPN). METHODS: A retrospective review of a prospective neurosurgical database at our institution was performed, 2000-2017, with a corresponding systematic literature review. Pain outcomes were dichotomized as unfavorable for unchanged/worsened symptoms versus favorable if improved/resolved. Eight formalin-fixed brains were examined to describe NI at the brainstem. RESULTS: Eleven patients were surgically treated for GN-9 primary, 2 reoperations. The median age was 48, 7 patients were female, and the median follow-up was 11 months (range 3-143). Seven had ≥ 2 probable cranial neuralgias. NI was sectioned in 9 and treated via microvascular decompression (MVD) in 2. Five patients underwent simultaneous treatment for TN (4 MVD; 1 rhizotomy) and 5 for GPN (3 MVD; 2 rhizotomy). Eleven reported symptomatic improvement (100%); 8 initially reported complete resolution (73%). Pain outcomes at last contact were favorable in 8 (73%)-all among the 9 primary operations (89% vs 0%, p = 0.054). Six prior series reported outcomes in 111 patients. CONCLUSIONS: GN is rare, and diagnosis is confounded by symptomatic overlap with TN/GPN. Directed treatment of all possible neuralgias improved pain control in almost all primary operations. Repeat surgery seems a risk factor for an unfavorable outcome. NI is adherent to superomedial VIII at the brainstem; the intermediate/cisternal portion is optimal for visualization and sectioning.

Geniculate neuralgia: clinical, radiologic, and intraoperative correlates.

OBJECTIVE: Geniculate neuralgia is an uncommon pain syndrome that can be severe and disabling and is difficult to diagnose. METHODS: The literature was reviewed for geniculate neuralgia, including anatomy, presentation, and treatment. A case illustration was presented that demonstrates the novel brainstem functional imaging findings for geniculate neuralgia. A 39-year-old man presented with a history of left "deep" ear pain within his ear canal. He noted occasional pain on the left side of his face around the ear. He had been treated with neuropathic pain medications without relief. His wife described suicidal ideations discussed by her husband because of the intense pain. RESULTS: The patient's neurologic examination was normal, and otolaryngologic consultation revealed no underlying structural disorder. Anatomic imaging revealed a tortuous vertebral artery-posterior inferior cerebellar artery complex with the posterior inferior cerebellar artery loop impinging on the root entry zone of the nervus intermedius-vestibulocochlear nerve complex and just inferior to the root entry zone of the facial nerve and a small anterior inferior cerebellar artery loop interposed between the cranial nerve VII-VIII complex and the hypoglossal and glossopharyngeal nerves. A left-sided retromastoid craniotomy was performed, and the nervus intermedius was transected. An arterial loop in contact with the lower cranial nerves at the level of the brainstem was mobilized with a polytetrafluoroethylene implant. CONCLUSIONS: The patient indicated complete relief of his preoperative pain after surgery. He has remained pain-free with intact hearing and balance.

The nervus intermedius: a review of its anatomy, function, pathology, and role in neurosurgery.

BACKGROUND: Geniculate neuralgia, although uncommon, can be a debilitating pathology. Unfortunately, a thorough review of this pain syndrome and the clinical anatomy, function, and pathology of its most commonly associated nerve, the nervus intermedius, is lacking in the literature. Therefore, the present study aimed to further elucidate the diagnosis of this pain syndrome and its surgical treatment based on a review of the literature. METHODS: Using standard search engines, the literature was evaluated for germane reports regarding the nervus intermedius and associated pathology. A summary of this body of literature is presented. RESULTS: Since 1968, only approximately 50 peer-reviewed reports have been published regarding the nervus intermedius. Most of these are single-case reports and in reference to geniculate neuralgia. No report was a review of the literature. CONCLUSIONS: Neuralgia involving the nervus intermedius is uncommon, but when present, can be life altering. Microvascular decompression may be effective as a treatment. Along its cisternal course, the nerve may be difficult to distinguish from the facial nerve. Based on case reports and small series, long-term pain control can be seen after nerve sectioning or microvascular decompression, but no prospective studies exist. Such studies are now necessary to shed light on the efficacy of surgical treatment of nervus intermedius neuralgia.

Atypic geniculate neuralgia: atypic anatomic correlation of cranial nerve roots and AICA.

Geniculate neuralgia is a rare cause of craniofacial pains. The anterior inferior cerebellar artery is the offending vessel which compress nervus intermedius in the patients with typical geniculate neuralgia. We report a patient whose pain was atypical for either geniculate neuralgia and trigeminal neuralgia. At operation the anterior inferior cerebellar artery was coursing with the nerves and was separated. After the decompression the pain resolved immediately.

Síndrome de Ramsay Hunt: relato de caso / Ramsay Hunt Syndrome: case report

A Síndrome de Ramsay Hunt é definida por paralisia facial periférica acompanhada de um rash eritematosoe vesicular no pavilhão auricular ou na boca, causado por herpes zoster. Comparado com a paralisia de Bell,os pacientes com Síndrome de Ramsay Hunt geralmente apresentam um quadro de paralisia mais grave e dificilmentese recuperam totalmente. Alguns estudos sugerem que o tratamento com aciclovir e predinisona possa melhorar o prognóstico, entretanto estudos randomizados ainda são necessários. Os autores descrevem um caso desta síndrome acompanhado de ceratopatia de exposição.

Ramsay Hunt Syndrome consists in facial peripheric paralysis associated with auricular or mouth rash andvesicles, caused by herpes zoster. Compared to Bell’s paralysis, the patients with Ramsay Hunt Syndrome usually present worse paralisy and hardly have a total recovery. Some studies suggest that acyclovir and prednisone treatment may improve the prognosis, but random control studies are still necessary. The authors describeone case of this syndrome with exposure keratopathy.

Correlation between MRI and operative findings in Bell's palsy and Ramsay Hunt syndrome.

PURPOSE: To investigate the correlation between gadolinium enhanced magnetic resonance image (MRI) results and surgical findings of facial nerves in Bell's palsy and Ramsay Hunt syndrome. MATERIALS AND METHODS: From 1995 to 2004, MRI was performed on 13 patients with Bell's palsy or Ramsay Hunt syndrome, who were offered with surgical decompression of the facial nerve through the middle cranial fossa approach. Gadolinium enhanced MRI was performed on all patients and the enhancement of the facial nerve was evaluated by radiology specialists. Operative findings including the degree of the facial nerve segment swelling were examined. Furthermore, the time interval from the onset of palsy to surgery was evaluated. RESULTS: Swelling of facial nerve segments was found in patients with enhanced facial nerves from MRI. The swelling of the facial nerve in the labyrinthine segment in particular was identified in all patients with enhanced labyrinthine segments in MRI. The intraoperative swelling of geniculate ganglion of facial nerve was found in 78% of patients with enhanced facial segment in MRI (p=0.01). The intraoperative swelling of tympanic segment was observed from fourth to ninth weeks after the onset of palsy. CONCLUSION: MRI enhancement of facial nerves in Bell's palsy and Ramsay Hunt syndrome is associated with the extent of intratemporal lesions of facial nerves, especially in the labyrinthine segment.

Prompt contrast enhancement of cerebrospinal fluid space in the fundus of the internal auditory canal: observations in patients with meningeal diseases on 3D-FLAIR images at 3 Tesla.

We speculated that meningeal pathologies might facilitate the permeability of cranial nerves at the fundus of the internal auditory canal (IAC), causing prompt enhancement after administration of Gd-DTPA. Using a 3D- fluid-attenuated inversion recovery (FLAIR) sequence, we evaluated the enhancement of the cerebrospinal fluid (CSF) space in the IAC fundus 10 min after Gd-DTPA administration in patients with meningeal diseases. Twenty patients (aged 22 to 79 years) were divided into 2 groups, a group with meningeal disease comprising 9 patients with meningeal abnormalities (6, tumor dissemination; 3, infection) and a control group of 11 patients with unilateral IAC pathology whose healthy sides were included as controls. Six of the 9 patients in the group with meningeal disease showed bilateral enhancement; one showed unilateral enhancement. None of the control group showed enhancement in the healthy side. One patient with Ramsay-Hunt syndrome showed only ipsilateral enhancement. Enhancement in the IAC fundus was frequently observed in patients with meningeal disease, even just 10 min after administration of contrast agent. This enhancement in the IAC fundus was never visible on T1-weighted 3D-FLASH images.

Síndrome de ramsay-hunt: a propósito de un case en el Hospital Militar "Cap. (AV)(F) Guilllermo Hernández Jacobsen" / Syndrome of ramsay-hunt: a purpose of a case in the Hospital Militar

El síndrome de Ramsay Hunt, es una causa infecciosa de parálisis del nervio facial, teniendo actualmente una incidencia de 15 a 40 casos por 100000 habitantes. El objetivo del presente trabajo, es dar a conocer esta patología así como establecer pautas de tratamiento. Realizamos una revisión bibliográfica, efectuando una actualización del mismo. La mayor incidencia esta en los 40 y 60 años, no encontrando distinción en la incidencia entre hombre y mujeres, generalmente existe remisión total de la enfermedad, este síndrome es el principal responsable de la parálisis de Bell. El tratamiento debe consistir en antivirales. Todo paciente portador de parálisis de Bell deberá ser revisado en busca de lesiones herpeticas que comprometan el trayecto inervado por el octavo y séptimo par craneal, así como esta indicado se le practiquen, pruebas inmunológicas, tales como la reacción de cadena de polimerasas para virus de la varicela-zoster, Debe ser tratado con antivirales, a dosis similares a las empleadas en la primo infección por virus del herpes, así como se incluirán esteroides para su tratamiento ya que la fisiopatología es debida al proceso inflamatorio. Siendo esta patología poco conocida por el personal de salud, nos proponemos a difundir nuestra actualización y resultados encontrados

Facial nerve palsy: evaluation by contrast-enhanced MR imaging.

AIM: The purpose of this study was to investigate the value of contrast-enhanced magnetic resonance (MR) imaging in patients with peripheral facial nerve palsy. MATERIALS AND METHODS: MR imaging was performed in 147 patients with facial nerve palsy, using a 1.0 T unit. All of 147 patients were evaluated by contrast-enhanced MR imaging and the pattern of enhancement was compared with that in 300 control subjects evaluated for suspected acoustic neurinoma. RESULTS: The intrameatal and labyrinthine segments of the normal facial nerve did not show enhancement, whereas enhancement of the distal intrameatal segment and the labyrinthine segment was respectively found in 67% and 43% of patients with Bell's palsy. The geniculate ganglion or the tympanic-mastoid segment was enhanced in 21% of normal controls versus 91% of patients with Bell's palsy. Abnormal enhancement of the non-paralyzed facial nerve was found in a patient with bilateral temporal bone fracture. CONCLUSION: Enhancement of the distal intrameatal and labyrinthine segments is specific for facial nerve palsy. Contrast-enhanced MR imaging can reveal inflammatory facial nerve lesions and traumatic nerve injury, including clinically silent damage in trauma.

Case report: prolonged contrast enhancement of the inner ear on magnetic resonance imaging in Ramsay Hunt syndrome.

There have been recent reports of enhancement of the inner ear in acute labyrinthitis on gadolinium enhanced magnetic resonance imaging (MRI). However, none has described persistence of enhancement beyond 6 weeks. We report a case of Ramsay Hunt syndrome with labyrinthitis, sensorineural hearing loss and facial nerve palsy in which marked enhancement of the inner ear structures was observed on MRI 6 months after the onset of symptoms. Enhancement on delayed or repeated imaging after a period of months does not exclude labyrinthitis from the differential diagnosis of the small intracanalicular acoustic neuroma, and care should be taken not to confuse them.

Gd-DTPA enhanced MRI in Ramsay Hunt syndrome.

Ten patients with Ramsay Hunt syndrome underwent magnetic resonance (MR) scans. In many examinations, abnormal enhancement of the 7th nerve in the internal acoustic meatal segment through the mastoid segment was observed. Out of seven patients with cochlear and/or vestibular symptoms, only one showed abnormal enhancement of the 8th nerve, in addition to the 7th. The other 6 patients showed the same findings as in Bell's palsy, showing no enhancement of the 8th nerve. This suggests that clinical symptoms have no relation to the results of MRI. Enhanced MRI is the most sensitive means of making differential diagnoses between Hunt's syndrome and tumors, but it is impossible to detect all lesion sites corresponding to the symptoms in Hunt's syndrome.