UpToDate: Intraneural ganglion cyst

An intraneural ganglion cyst (INGC) is a non-neoplastic mucinous cyst within the epineurium of a nerve and commences from an adjoining joint 1) 2)3) 4) 5) 6) 7).

These cysts are filled with a mucinous material which is walled off by a fibrous layer 8) 9) 10)

An intraneural ganglion cyst is an uncommon occurrence of the peripheral nerves.

Types

The most common type is the peroneal intraneural ganglion cyst. Other reported sites of involvement are the radial, ulnar, median, sciatic, tibial, and posterior interosseus nerves. The first case of intraneural ganglion cyst of the tibial nerve was described in 1967.

Etiology

According to the most widely accepted theory (articular/synovial theory), the cysts are formed from a capsular defect of an adjacent joint, so that synovial fluid spreads along the epineurium of a nerve branch 11).

Clinical features

As these cysts expand within the epineurium, they displace and compress the adjacent nerve fascicles leading to pain, paresthesia, tingling and muscle paralysis in the distribution of the involved nerve 12) 13).

Diagnosis

MRI is the method of choice for diagnosing intraneural ganglion cysts. However, ultrasound is also important 14).

Differential diagnosis

The differential considerations for cystic intraneural lesions include cystic nerve sheath tumors, atypical Baker’s cyst, and extraneural ganglion.

Cystic nerve sheath tumors such as schwannomas and extraneural ganglion can be differentiated from cystic intraneural lesions by MRI. A Baker’s cyst classically is more mass-like, with a characteristic location extending from the tibiofemoral joint to within the confines of the medial head of the gastrocnemius and the muscles of the joint capsule 15).

Treatment

Surgery is the only curative treatment with treatment success being dependent on ligature of the nerve endings supplying the articular branch 16).

Case series

Fricke et al. from Kiel, examined between 2011 and 2018 the patients using lower limb MRI. MRI scans were also performed for the follow-up examinations.

The patients had many symptoms. They were able to accurately detect the intraneural ganglion cysts on MRI and provide the treating surgeons with the basis for the operation to be performed.

The success of surgical therapy depends on the resection of the nerve endings supplying the joint as the only way to treat the origin of the disease and prevent recurrence. Based on there case studies, they can support the commonly favored articular/synovial theory. 17).

References

1) , 8)

Patel P, Schucany WG. A rare case of intraneural ganglion cyst involving the tibial nerve. Proc (Bayl Univ Med Cent) 2012;25:132–135.

2) , 9)

Uetani M, Hashmi R, Hayashi K, Nagatani Y, Narabayashi Y, Imamura K. Peripheral nerve intraneural ganglion cyst: MR findings in three cases. J Comput Assist Tomogr. 1998;22:629–632.

3) , 10)

Harbaugh KS, Tiel RL, Kline DG. Ganglion cyst involvement of peripheral nerves. J Neurosurg. 1997;87:403–408.

4)

Spinner RJ, Desy NM, Rock MG, Amrami KK. Peroneal intraneural ganglia. Part I. Techniques for successful diagnosis and treatment. Neurosurg Focus. 2007;22:E16.

5)

Jacobs RR, Maxwell JA, Kepes J. Ganglia of the nerve. Presentation of two unusual cases, a review of the literature, and a discussion of pathogenesis. Clin Orthop Relat Res. 1975:135–144.

6)

Adn M, Hamlat A, Morandi X, Guegan Y. Intraneural ganglion cyst of the tibial nerve. Acta Neurochir (Wien) 2006;148:885–889; discussion 889-890.

7)

Johnston JA, Lyne DE. Intraneural ganglion cyst of the peroneal nerve in a four-year-old girl: a case report. J Pediatr Orthop. 2007;27:944–946.

11) , 14) , 16) , 17)

Fricke T, Schmitt AD, Jansen O. Intraneural ganglion cysts of the lower limb. Rofo. 2018 Nov 19. doi: 10.1055/a-0777-2525. [Epub ahead of print] English, German. PubMed PMID: 30453381.

12)

Tehli O, Celikmez RC, Birgili B, Solmaz I, Celik E. Pure peroneal intraneural ganglion cyst ascending along the sciatic nerve. Turk Neurosurg. 2011;21:254–258.

13)

Liang T, Panu A, Crowther S, Low G, Lambert R. Ultrasound-guided aspiration and injection of an intraneural ganglion cyst of the common peroneal nerve. HSS J. 2013;9:270–274.

15)

Patel P, Schucany WG. A rare case of intraneural ganglion cyst involving the tibial nerve. Proc (Bayl Univ Med Cent). 2012 Apr;25(2):132-5. PubMed PMID: 22481843; PubMed Central PMCID: PMC3310510.
intraneural_ganglion_cyst.txt · Last modified: 2018/11/20 20:09 by administrador

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UpToDate: Mohr-Tranebjaerg Syndrome

Mohr-Tranebjaerg Syndrome

Deafnessdystoniaoptic neuronopathy (DDON) syndrome, also known as Mohr-Tranebjærg syndrome, is characterized by hearing loss that begins early in life, problems with movement, impaired vision, and behaviorproblems. This condition occurs almost exclusively in males.

Case reports

Coenen et al. from the Department of Stereotactic and Functional Neurosurgery, Department of Neurology and Neurophysiology, Department of Neuroradiology, University Hospital Freiburg and Parkinson-Klinik Wolfach, Germany, reported a 28-year-old man presented with a history of sensorineural deafness since early childhood treated with bilateral cochlear implants (CIs). He showed signs of debilitating dystonia that had been present since puberty. Dystonic symptoms, especially a protrusion of the tongue and bilateral hand tremor, had not responded to botulinum toxin therapy. They diagnosed Mohr-Tranebjaerg syndrome (MTS).

Deep brain stimulation (DBS) of the bilateral globus pallidus internus was performed predominantly with stereotaxic computed tomography angiography guidance under general anesthesiaElectrophysiology was used to identify the target regions and to guide DBS electrode placement.

In the immediate postoperative course and stimulation, the patient showed marked improvement of facial, extremity, and cervical dystonia. More than 2 years after implantation, his dystonic symptoms had dramatically improved by 82%.

The use of DBS for the dystonia in MTS was previously described but not in the presence of bilateral CIs.

DBS in MTS may be a viable option to treat debilitating dystonic symptoms. They describe successful DBS surgery, despite the presence of bilateral CIs, and stimulation therapy over 2 years 1).


Eggink et al. from the Department of Neurology, Department of Genetics, Department of Rehabilitation, Department of Neurosurgery, University Medical Center Groningen, The Netherlands, reported two patients with dystonia-deafness syndrome due to a beta-actin gene mutation.

They report on disease course, genetic testing, and management of 2 patients, mother and daughter, presenting with dystonia-deafness syndrome.

After exclusion of known dystonia-deafness syndrome causes, whole-exome sequencing revealed a beta-actin gene mutation (p.Arg183Trp) in both patients. Although beta-actin gene mutations are generally associated with developmental Baraitser-Winter syndrome, dystonia-deafness syndrome has been reported once in identical twin brothers. Bilateral GPi-DBS led to a significant decrease of dystonia and regain of independency in our patients.

The p.Arg183Trp mutation in the beta-actin gene is associated with the clinical presentation of dystonia-deafness syndrome, even with only minimal or no developmental abnormalities of Baraitser-Winter syndrome. GPi-DBS should be considered to ameliorate the invalidating dystonia in these patients. 2).


Cif et al. reported in 2013 the article Progressive dystonia in Mohr-Tranebjaerg syndrome with cochlear implant and deep brain stimulation 3).

References

1)

Coenen VA, Rijntjes M, Sajonz B, Piroth T, Prokop T, Jost W, Trippel M, Urbach H, Reinacher PC. Bilateral Globus Pallidus Internus Deep Brain Stimulation in a Case of Progressive Dystonia in Mohr-Tranebjaerg Syndrome with Bilateral Cochlear Implants. J Neurol Surg A Cent Eur Neurosurg. 2018 Oct 5. doi: 10.1055/s-0038-1669472. [Epub ahead of print] PubMed PMID: 30290379.

2)

Eggink H, van Egmond ME, Verschuuren-Bemelmans CC, Schönherr MC, de Koning TJ, Oterdoom DL, van Dijk JM, Tijssen MA. Dystonia-deafness syndrome caused by a β-actin gene mutation and response to deep brain stimulation. Mov Disord. 2017 Jan;32(1):162-165. doi: 10.1002/mds.26842. Epub 2016 Nov 8. PubMed PMID: 27862284.

3)

Cif L, Gonzalez V, Garcia-Ptacek S, James S, Boetto J, Seychelles A, Roujeau T, Moura De Ribeiro AM, Sillon M, Mondain M, Coubes P. Progressive dystonia in Mohr-Tranebjaerg syndrome with cochlear implant and deep brain stimulation. Mov Disord. 2013 Jun;28(6):737-8. doi: 10.1002/mds.25519. PubMed PMID: 23801560.

UpToDate: Combined Unilateral Posteroventral Pallidotomy and Ventral Intermediate Nucleus Thalamotomy

Combined Unilateral Posteroventral Pallidotomy and Ventral Intermediate Nucleus Thalamotomy

Patients with tremor predominant Parkinson’s disease (PD) achieve more improvement in tremor control after combined unilateral posteroventral pallidotomy and ventral intermediate nucleus thalamotomy 1)2).

Case series

Twenty-four patients with tremor-dominant PD were included in a study of Fayed et al. from the Department of Neurosurgery, Faculty of Medicine, Ain Shams University, CairoEgypt.

Twelve patients received unilateral PVP contralateral to the most affected side. The other 12 patients received simultaneous unilateral PVP and VIM thalamotomy contralateral to the most affected side. Assessment of results in both groups was achieved using both UPDRS “off” motor scores and UPDRS rest tremor subscores.

The mean UPDRS off motor score improved in the pallidotomy group from 61.3 preoperatively to 36.8 at 12 months. In the combined group, it improved from 59.6 to 35.2 at 12 months, with no statistically significant difference between both groups. On the other hand, while the mean tremor subscore in the pallidotomy group improved from a mean of 2.3-0.8, the tremors were abolished in all of the patients in the combined group except for 1 patient who showed slight infrequent tremors at 12 months 3).


Iacono et al. from the Division of Neurosurgery, Loma Linda University Medical Center, combined Vim/VOp junction thalamotomy and PVP in 29 patients with severe tremorrigidity, and bradykinesia. Patients underwent unilateral Vim thalamotomy followed at the same sitting by PVP. The distinct physiological consequences of each procedure were documented by intraoperative electromyography (EMG) and video recording, revealing the effects on both tremor and agonist/antagonist co-contraction. Lack of reciprocal inhibition of antagonistic muscle groups often remained following thalamotomy but was eliminated by subsequent PVP. The complementary therapeutic effects of PVP and Vim thalamotomy may be due to the interruption of different neuronal circuits by the two procedures. The effect of Vim thalamotomy has been attributed to the interruption of the rubrothalamocortical loop. PVP interrupts the outflow of the globus pallidus internus (GPi), which may cause disinhibition of locomotor centers in the mesencephalon and spinal cord. There is no direct interruption of the rubrothalamocortical loop by PVP, explaining why this procedure sometimes exacerbates tremor in certain patients 4).

References

1) , 3)

Fayed ZY, Radwan H, Aziz M, Eid M, Mansour AH, Nosseir M, Anwer H, Elserry T, Abdel Ghany WA. Combined Unilateral Posteroventral Pallidotomy and Ventral Intermediate Nucleus Thalamotomy in Tremor-Dominant Parkinson's Disease versus Posteroventral Pallidotomy Alone: A Prospective Comparative Study. Stereotact Funct Neurosurg. 2018 Sep 18;96(4):1-6. doi: 10.1159/000492229. [Epub ahead of print] PubMed PMID: 30227440.

2) , 4)

Iacono RP, Henderson JM, Lonser RR. Combined stereotactic thalamotomy and posteroventral pallidotomy for Parkinson’s disease. J Image Guid Surg. 1995;1(3):133-40. PubMed PMID: 9079438.
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