31/37. Correlation of MRI, clinical, and electroneuronographic findings in acute facial nerve palsy. Intratemporal enhancement of (Gd-DTPA) was investigated by an interleaved-overlapping magnetic resonance imaging (MRI) technique in 35 cases of acute facial palsy. In a reference group (normal facial function), enhancement was localized from the ganglion geniculi to the stylomastoid foramen. In cases of acute palsy, the facial nerve enhanced in the meatal fundus independent of etiology (idiopathic, herpetic, or traumatic). In 70% of those with Ramsay-Hunt syndrome, the vestibular and cochlear nerves, the labyrinth, and the sheets of the internal and external auditory canal additionally enhanced. No correlation was found between intensity, extension, and duration of the enhancement and the clinical, intraoperative, or electroneuronographic degree of the facial palsy. The pathogenesis of the Gd-DTPA enhancement of the facial nerve appears to be closely connected with the vascular supply of the fallopian canal and the permeability of the neural sheets. ( info) |
32/37. Ramsay Hunt syndrome presenting as a cranial polyneuropathy. Ramsay Hunt syndrome is herpes zoster of the facial nerve, frequently associated with VIII cranial nerve involvement, but on rare occasions V, VI, IX, and X cranial nerves are affected as well. We present a case of a Ramsay Hunt syndrome with involvement of V, VII, and VIII cranial nerves. ( info) |
| Varicella-zoster virus has been associated with a variety of neurological manifestations. We describe a patient with the Ramsay Hunt syndrome who developed a contralateral cerebral infarction. ( info) |
34/37. Cranial polyneuropathy--Ramsay Hunt's syndrome: case report and discussion. Ramsay Hunt's syndrome is an infectious cranial polyneuropathy caused by varicella zoster, the herpetic virus that also causes chickenpox and shingles. Its symptoms include facial paralysis, ear pain, and an auricular rash. Oral lesions are also present in most cases. This syndrome can affect any cranial nerve and usually affects multiple nerves, causing central, cervical, and peripheral effects. This article reports the case of a 35-year-old white female who was treated by the oral surgery service of a large urban hospital, after first reporting to the emergency clinic. Her reported symptoms of unilateral left-side facial paralysis, auricular pain, and trigeminal hyperesthesia were confirmed by clinical examination. An initial short low-dose steroid regimen was unsuccessful. A second daily dosage of 50 mg of prednisone was successful in 21 days. No permanent sequelae were evident or reported after treatment. ( info) |
35/37. Surgical management of geniculate neuralgia. BACKGROUND: geniculate ganglion or nervus intermedius neuraigia is an unusual condition resulting in deep ear pain with or without signs of atypical trigeminal neuralgia, deep face, or throat pain. This article describes an experience with 14 patients who came to the neurosurgical service at the University of Pittsburgh Medical Center with a diagnosis of geniculate neuralgia. methods: After failing conservative treatment and after undergoing neurologic, otologic, and dental evaluations, these 14 patients underwent 20 intracranial procedures consisting of retromastoid craniectomies with microvascular decompression of cranial nerves V, IX, and X with section of the nervus intermedius in most cases. RESULTS: At operation, vascular compression of the nerves and nervus intermedius was found, which implicated vascular compression as an etiology of this disorder. Initially, 10 of 14 patients had an excellent outcome (71.5%), 3 experienced partial relief (21.5%), and there was 1 failure (7%). Ten patients were available for long-term (> 12 months) follow-up. Of these 10, 3 retained the excellent result (30%), 6 experienced partial relief (60%), and there was 1 failure (10%). Complications included one transient facial paresis, one facial numbness, one paresis of cranial nerves IX and X, one chemical meningitis, two cerebrospinal fluid leaks, and one superficial wound infection. Of those that fell from the excellent to partial category, this usually involved a return of atypical facial pain, but otalgia remained resolved. CONCLUSIONS: overall, good results (with excellent or partial relief) were found long term for 90% of patients in this series. The authors recommend microvascular decompression of cranial nerves V, IX, and X with nervus intermedius section for the treatment of geniculate neuralgia. ( info) |
| In 1907 J. Ramsay Hunt suggested that herpes zoster oticus resulted from a geniculate ganglionitis; however, many contemporary authors believe that this disorder represents a neuritis or polycranial neuropathy. Herpes varicella-zoster viral (VZV) dna was identified, using the polymerase chain reaction, in archival celloidin-embedded temporal bone sections from two patients who clinically had Ramsay Hunt syndrome (herpes zoster oticus). The presence of VZV was confirmed by sequencing the PCR products. These experiments demonstrated that VZV genomic dna was present in the geniculate ganglion of the side with facial paralysis and cutaneous recrudescence in both patients and in the clinically unaffected side in patient 1. In addition, patient 2 had a sudden hearing loss and was found to have VZV genomic dna in sections from the affected side containing the spiral ganglion, Scarpa's ganglion, organ of corti, and macula of the saccule. No VZV genomic dna was identified in temporal bone sections from five patients with Bell's palsy and ten patients without evidence of otologic disease. In this study, the histopathology of these two cases yielded complementary information regarding the role of VZV in herpes zoster oticus. These data suggest that in patients with Ramsay Hunt syndrome, latent VZV is located in the geniculate ganglia and may be present in the auditory and vestibular primary afferent ganglia in some patients. ( info) |
37/37. herpes zoster oticus: correlations between clinical and MRI findings. Many gadolinium-enhanced magnetic resonance imaging (MRI) studies focusing on the anatomy and pathology of the 7th cranial nerve have already been published. However, only scattered cases of herpes zoster oticus (HZO) have been described and only the MRI appearance of the soft temporal bone structures has been reported. Enhanced MRI was performed in 4 patients with HZO observed at the Department of Otorhinolaryngology of the University of Pisa. A good correlation was found between the clinical data and MRI findings in both the acute and chronic stages of the disease. The 2 cases with complete facial palsy presented prominent and diffuse enhancement of the 7th and 8th cranial nerves on postcontrast MRI, while the patient with grade III facial palsy showed more limited nerve enhancement. The patient with grade II facial palsy presented no MRI abnormalities. In our series, enhancement limited to the geniculate ganglion and to the labyrinthine segment of the facial nerve indicates a good prognosis while a widespread enhancement correlates with a poor prognosis. In conclusion, MRI with contrast may be useful during the acute stage of HZO because it can confirm the diagnosis and can provide prognostic information on the facial function. ( info) |