Raeder's Syndrome. A Case With an Unusual Localization

Cephalalgia ◽  
1993 ◽  
Vol 13 (2) ◽  
pp. 135-135 ◽  
Author(s):  
José Pimentel ◽  
Isabel Pavão Martins
Keyword(s):  
Cavernous Sinus ◽  
Trigeminal Nerve ◽  
Sensory Loss ◽  
Sweat Glands ◽  
Orbital Pain

We describe a patient with a Raeder's paratrigeminal neuralgia consisting of left-sided frontal and orbital pain, Homer syndrome (including anhydrosis of the forehead), and sensory loss in the territory of the first division of the trigeminal nerve. The involvement of the ophthalmic nerve is not consistent with the usual localization of this syndrome to the pericarotid sympathetic plexus. Oculosympathetic and sympathetic fibers supplying the sweat glands of the forehead join the ophthalmic nerve in the cavernous plexus localized in the cavernous sinus. Therefore, this seems to be the most likely site of the lesion when the ophthalmic nerve is involved.

scholarly journals Tolosa-Hunt Syndrome: A Case Report and Review of the Literature

2013 ◽  
Vol 29 (2) ◽  
pp. 130-133
Author(s):  
Md Rafiqul Islam ◽  
Hasan Zahidur Rahman ◽  
Akm Anwar Ullah ◽  
Md Amir Hossain ◽  
- Md Ziauddin
Keyword(s):  
Cavernous Sinus ◽  
Trigeminal Nerve ◽  
Cranial Nerve ◽  
Motor Nerve ◽  
Pathological Process ◽  
Sensory Loss ◽  
Oral Steroid ◽  
Superior Orbital Fissure ◽  
Hunt Syndrome ◽  
Cranial Nerve Palsies

Tolosa-Hunt Syndrome is a painful ophthalmoplegia which is characterized by periorbital or hemicranial pain, with ipsilateral ocular motor nerve palsies, oculosympathetic paralysis, sensory loss in the distribution of the ophthalmic and occasionally the maxillary division of the trigeminal nerve. Various combinations of these cranial nerve palsies may occur, localising the pathological process to the region of the cavernous sinus/superior orbital fissure. We report the case of a patient presented with severe pain in the right side of face which was periorbital with ipsilateral 3rd,4th, 6th cranial nerve palsies along with ophthalmic and maxillary division of trigeminal nerve involvement. MRI of orbit showed hypo-intense lesion in right cavernous sinus extending to right superior orbital fissure (suggestive of granulomatous infiltration). After taking oral steroid her pain was relieved quickly and cranial nerve palsies reversed within one week. Azathioprin was added and she was completely cured of within next three months. Bangladesh Journal of Neuroscience 2013; Vol. 29 (2) : 130-133

scholarly journals Trigeminal Nerve Schwannoma of the Cerebellopontine Angle

2018 ◽  
Vol 79 (S 05) ◽  
pp. S389-S390
Author(s):  
Maria Peris-Celda ◽  
Christopher Graffeo ◽  
Avital Perry ◽  
Lucas Carlstrom ◽  
Michael Link
Keyword(s):  
Trigeminal Nerve ◽  
Cerebellopontine Angle ◽  
Incidental Finding ◽  
Cranial Nerves ◽  
Internal Auditory Canal ◽  
Clinical History ◽  
Sensory Loss ◽  
Meckel’S Cave ◽  
Neurologic Deficits ◽  
Meckel's Cave

Introduction Large and even moderate sized, extra-axial cerebellopontine angle (CPA) tumors may fill this restricted space and distort the regional anatomy. It may be difficult to determine even with high resolution magnetic resonance imaging (MRI) if the tumor is dural-based, or what the nerve of origin is if a schwannoma. While clinical history and exam are helpful, they are not unequivocal, particularly since many patients present with a myriad of symptoms, or conversely an incidental finding. We present an atypical appearing, asymptomatic CPA tumor, ultimately identified at surgery to be a trigeminal schwannoma. Case History A 40-year-old man presented with new-onset seizure. MRI identified an incidental heterogeneously contrast-enhancing CPA lesion (Fig. 1A–D). The tumor was centered on the internal auditory canal (IAC) with no tumor extension into Meckel's cave, IAC or jugular foramen. Audiometry demonstrated 10db of relative left-sided hearing loss with 100% word recognition. Physical examination was negative for focal neurologic deficits. A retrosigmoid craniotomy was performed and an extra-axial, yellow-hued mass was encountered and resected, which was ultimately confirmed to originate from the trigeminal nerve (Video 1). Gross total resection was achieved, and the patient recovered from surgery with partial ipsilateral trigeminal sensory loss and no other new neurologic deficits. Conclusion Pure CPA trigeminal schwannomas are rare, but should be considered in the differential for enhancing CPA lesions. Although, Meckel's cave involvement is frequently observed, it is not universal, and pure CPA schwannomas of all cranial nerves IV–XII have been reported in the literature.The link to the video can be found at: https://youtu.be/AlodYCu70F8.

A juvenile case of idiopathic hypertrophic pachymeningitis involved cavernous sinus and proximal trigeminal nerve

2019 ◽  
Vol 8 (2) ◽  
pp. 82-85
Author(s):  
Zhihong Bian ◽  
Yoshio Omote ◽  
Koh Tadokoro ◽  
Ken Ikegami ◽  
Yosuke Osakada ◽  
...  
Keyword(s):  
Cavernous Sinus ◽  
Trigeminal Nerve ◽  
Hypertrophic Pachymeningitis

scholarly journals Trigeminal Amyloidoma: A Report of Two Cases and Review of the Literature

2019 ◽  
Vol 81 (06) ◽  
pp. 620-626
Author(s):  
Amy Swanson ◽  
Caterina Giannini ◽  
Michael Link ◽  
Jamie Van Gompel ◽  
John Wald ◽  
...  
Keyword(s):  
Mass Spectrometry ◽  
Trigeminal Nerve ◽  
Systemic Disease ◽  
Protein Characterization ◽  
The Other ◽  
Sensory Loss ◽  
Meckel’S Cave ◽  
Extensive Evaluation ◽  
Amyloid Deposits ◽  
Meckel's Cave

AbstractCerebral amyloidomas, characterized by localized amyloid deposits in the nervous system in the absence of systemic disease, are rare. These typically consist of immunoglobulin light chain (AL)-type, predominantly lambda. Trigeminal nerve involvement is exceptionally rare with only 21 previously reported cases, three with bilateral disease. We report two additional cases of amyloid localized to Meckel’s cave with secondary involvement of the trigeminal nerves bilaterally, with protein characterization by mass spectrometry. The patients, both females, 39 and 49-years-old, respectively, presented with the insidious onset of progressive trigeminal neuropathy, including pain and numbness with sensory loss, refractory to medical therapy. One patient experienced bilateral symptoms. Magnetic resonance imaging demonstrated abnormal thickening and contrast enhancement along Meckel’s cave bilaterally in both cases. The clinical differential diagnosis included benign neoplasms and inflammatory disorders. At the time of biopsy, the trigeminal nerve was noted to be enlarged and multinodular in one case and associated with abnormal soft tan tissue in the other case. Microscopically, the nerve biopsies showed extensive Congo red-positive amyloid deposits. Liquid chromatography tandem mass spectrometry demonstrated that the amyloid was of (AL)-type in both cases (AL [kappa] in one case and AL [lambda] in the other). After extensive evaluation, there was no evidence of systemic involvement. Both patients received localized radiotherapy for their refractory symptoms. One patient has stable symptomatology and imaging. No follow-up is available for the other patient.

Isolated Trigeminal Nerve Sarcoid Granuloma Mimicking Trigeminal Schwannoma: Case Report

Neurosurgery ◽  
2003 ◽  
Vol 52 (3) ◽  
pp. 700-705 ◽  
Author(s):  
Alfredo Quinones-Hinojosa ◽  
Edward F. Chang ◽  
Saad A. Khan ◽  
Michael W. McDermott
Keyword(s):  
Cavernous Sinus ◽  
Trigeminal Nerve ◽  
Facial Pain ◽  
Frozen Section ◽  
Cranial Nerves ◽  
Lateral Wall ◽  
Neurophysiological Monitoring ◽  
Pathology Report ◽  
Trigeminal Schwannoma ◽  
Sarcoid Granuloma

Abstract OBJECTIVE AND IMPORTANCE Sarcoidosis most commonly presents as a systemic disorder. Infrequently, sarcoidosis can manifest itself in the central nervous system, with granulomas involving the leptomeninges and presenting with facial nerve weakness. Sarcoid of the trigeminal nerve is exceedingly rare and can mimic trigeminal schwannoma. We review the literature on sarcoid granulomas of the trigeminal nerve and compare their radiological features with the more common schwannoma. CLINICAL PRESENTATION A 33-year-old woman presented with a history of left-sided facial pain and numbness for 11 months, which was presumed to be trigeminal neuralgia. A trial of carbamazepine had been unsuccessful in relieving the facial pain. Her neurological examination revealed decreased facial sensation in the V1–V2 distribution. Preoperative magnetic resonance imaging demonstrated a contrast-enhancing mass centered in the left cavernous sinus with extension along the cisternal portion of the left trigeminal nerve. INTERVENTION The patient underwent a left frontotemporal orbitozygomatic craniotomy with intraoperative neurophysiological monitoring of Cranial Nerves III, V, and VI and image guidance for subtotal microsurgical resection of what appeared, grossly and on frozen section, to be a neurofibroma. The final pathology report, however, revealed a sarcoid granuloma of the trigeminal nerve. CONCLUSION The differential diagnosis of contrast-enhancing lesions in the lateral wall of the cavernous sinus should include inflammatory conditions such as sarcoidosis. We recommend that surgery for biopsy or decompression be used only for those patients in whom a diagnosis cannot be confirmed with noninvasive testing. If surgery is performed, intraoperative frozen pathology is very useful in guiding the extent of resection.

scholarly journals Ophthalmoplegia and cranial nerve deficits in an adolescent with headache

2021 ◽  
Vol 9 ◽  
pp. 2050313X2110244
Author(s):  
Margarita M Corredor ◽  
Peter J Holmberg
Keyword(s):  
Cavernous Sinus ◽  
Cranial Nerve ◽  
Cranial Nerves ◽  
Signs And Symptoms ◽  
Inflammatory Condition ◽  
Cranial Nerve Involvement ◽  
Hunt Syndrome ◽  
Nerve Involvement ◽  
Orbital Pain

Tolosa–Hunt syndrome is an idiopathic, inflammatory condition involving the cavernous sinus and is characterized by unilateral, painful ophthalmoparesis. The condition often begins with retro-orbital pain followed by select cranial nerve involvement. We report the case of a 17-year-old female whose presentation with progressive left-sided headache and ophthalmoparesis culminated in the diagnosis of Tolosa–Hunt syndrome. While many of her signs and symptoms have been previously reported in the rare pediatric cases of Tolosa–Hunt syndrome described in the literature, this case illustrates a unique presentation involving cranial nerves V and VII in addition to the more commonly reported cranial nerve III, IV, and VI palsies.

A Comparison of Percutaneous Radiofrequency Trigeminal Neurolysis and Microvascular Decompression of the Trigeminal Nerve for the Treatment of Tic Douloureux

Neurosurgery ◽  
1977 ◽  
Vol 1 (1) ◽  
pp. 16-21 ◽  
Author(s):  
Ronald I. Apfelbaum
Keyword(s):  
Trigeminal Neuralgia ◽  
Trigeminal Nerve ◽  
Microvascular Decompression ◽  
Sensory Loss ◽  
Tic Douloureux ◽  
Suboccipital Craniectomy ◽  
Early Results ◽  
Classical Trigeminal Neuralgia

Abstract In a 36-month period, 103 consecutive patients have been treated for classical trigeminal neuralgia with either percutaneous radiofrequency trigeminal neurolysis (PTN) (48 patients) or microvascular decompression (MVD) via a suboccipital craniectomy (55 patients). The results of these two procedures are tabulated, emphasizing especially the complications that have occurred with each. Successful initial relief of pain was achieved in 88% of the patients with PTN and 96% of the patients with MVD. Two significant complications occurred in the former group. Severe recurrences have occurred to date in 13% of the patients with PTN and in 5% of those with MVD. It is concluded that both procedures are effective, but that microvascular decompression offers the advantage of avoiding sensory loss and associated dysesthetic sensations. Follow-up is too short to conclude that MVD is a curative procedure, but the early results are very encouraging.

scholarly journals Tolosa-Hunt syndrome in pediatric practice

2020 ◽  
Vol 3 (4) ◽  
pp. 336-339
Author(s):  
E.V. Shishkina ◽  
◽  
T.N. Bazilevskaya ◽  
R.F. Izokhvatova ◽  
Yu.V. Panfilova ◽  
...  
Keyword(s):  
Cavernous Sinus ◽  
Clinical Signs ◽  
Granulomatous Inflammation ◽  
Helical Ct ◽  
Lateral Wall ◽  
Pulse Therapy ◽  
Careful Examination ◽  
Pediatric Practice ◽  
Hunt Syndrome ◽  
Orbital Pain

Tolosa-Hunt syndrome (THS) is a rare disease manifested with one or more cranial nerve disorders and orbital pain. In children, THS is diagnosed very rarely due to the lack of vigilance among physicians. Careful examination including MRI or helical CT help diagnose this disease. This paper discusses case history of THS in a child in the first ten years of life who has previously experienced similar episodes of ptosis and right-sided orbital pain; however, the cause of these symptoms has remained elusive. Disease anamnesis, clinical signs, neurological status, and additional diagnostic data are described in detail. Clinical course of THS with its relapses and remissions, rapid symptom regress after corticosteroid pulse therapy, and no specific granulomatous inflammation of the lateral wall of the cavernous sinus as demonstrated by MRI suggest idiopathic THS. The importance of in-depth neurological and general examination of patients with clinical signs of THS due to a variety of the causes of this syndrome is highlighted. KEYWORDS: pediatrics, Tolosa-Hunt syndrome, ophthalmoplegia, superior orbital fissure, cavernous sinus, corticosteroids. FOR CITATION: Shishkina E.V., Bazilevskaya T.N., Izokhvatova R.F. et al. Tolosa-Hunt syndrome in pediatric practice. Russian Journal of Woman and Child Health. 2020;3(4):336–339. DOI: 10.32364/2618-8430-2020-3-4-336-339.

scholarly journals Identification of a Persistent Primitive Trigeminal Artery Following the Transposition Technique for Trigeminal Neuralgia: A Case Report

2011 ◽  
Vol 16 (5) ◽  
pp. 357-359 ◽  
Author(s):  
Naoki Kato ◽  
Toshihide Tanaka ◽  
Hiroki Sakamoto ◽  
Takao Arai ◽  
Yuzuru Hasegawa ◽  
...  
Keyword(s):  
Magnetic Resonance Imaging ◽  
Trigeminal Neuralgia ◽  
Trigeminal Nerve ◽  
Trigeminal Artery ◽  
Resonance Imaging ◽  
Entry Zone ◽  
Root Entry Zone ◽  
Persistent Primitive Trigeminal Artery ◽  
Orbital Pain ◽  
Primitive Trigeminal Artery

Trigeminal neuralgia is lancinating pain of a few seconds duration triggered by minor sensory stimuli such as speaking, chewing or even a breeze on the face. Vascular compression of the trigeminal nerve at the root entry zone and other vessels has been implicated in its cause. Despite the initial success of medical treatment in some cases, however, many patients become refractory over time and eventually require surgical intervention. This report describes a case involving a 62-year-old woman who presented with right orbital pain provoked by, among others, exercise and cold. Medication proved to be ineffective and, after magnetic resonance imaging, microvascular decompression and surgical observation, the diagnosis became clearer. The case highlights the importance of preoperative imaging and careful intraoperative findings to determine whether variant arteries are responsible for trigeminal neuralgia.A patient who presented with trigeminal neuralgia associated with a persistent primitive trigeminal artery (PPTA) is presented. A 62-year-old woman suffering from right orbital pain was admitted to the hospital. Medical treatment for three months was ineffective, and her neuralgia had deteriorated and gradually spread in the maxillary division. Magnetic resonance imaging demonstrated the flow void signal attached to the right trigeminal nerve. Thus, microvascular decompression was performed. The superior cerebellar artery was the responsible artery, and it was transposed to decompress the trigeminal nerve. After this manoeuvre, an artery was identified running parallel to the trigeminal nerve toward Meckel’s cave. The artery, which turned out to be a PPTA, communicated with the basilar artery. The PPTA was carefully observed, and it was found not to be the artery causing the neuralgia because it did not compress the nerve at surgical observation. No additional procedure between the PPTA and the trigeminal nerve was performed. The patient’s symptom improved dramatically following surgery, and her postoperative course was uneventful. Postoperative three-dimensional computed tomography showed the PPTA. The findings in the present case suggest that transposition of the responsible artery effectively decompresses the root entry zone and assists in determining whether the PPTA is affecting the trigeminal nerve.

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