Idiopathic normal pressure hydrocephalus outcome

Idiopathic normal pressure hydrocephalus outcome

Mild preoperative Idiopathic normal pressure hydrocephalus severity, shorter preoperative symptom duration, good tap test response, and complete disproportionately enlarged subarachnoid space hydrocephalus (DESH) were associated with good short-term postoperative outcome at 1 year. These positive factors may be useful for prediction of short-term surgical outcome in iNPH patients 1)


Comorbid conditions interfere with the ability to assess progression of iNPH and the effectiveness of the shunt. Patient caregivers play a large role in decision-making and clinical course, and should be included when counseling patients 2).

A 2001 meta-analysis of outcomes reported the Idiopathic normal pressure hydrocephalus treatment to have a 29% rate of significant improvement and a 6% significant complication rate 3).

A study in 2005 revealed greater improvements, with 75% of patients (n = 132) seeing postoperative improvements within 24 months of surgery 4), 68% of patients experiencing “very good” or “good” outcomes in a prospective study 5), and 69%–84% of patients seeing improvements by 1 year after surgery in a prospective multicenter study 6).

Studies that have established fixed protocols for follow-up have shown that short- and long-term periods after shunting are determined by many factors. Whereas short-term results were more likely to be influenced by shunt-associated risks, long-term results were independent of factors inherent to the shunt procedure and shunt complications, i.e., death and morbidity related to concomitant cerebrovascular and vascular diseases 7).

In 2013 a total of 64 studies of 3,063 patients were reviewed. Positive improvement following shunt insertion was reported in an average of 71 % of patients with an average 1 % mortality. Results from studies published in the last 5 years showed 82 % improvement following shunt insertion, mortality of 0.2 %, and combined common complications rate of 8.2 % 8).

Analysis of the efficacy of shunts for possible iNPH conducted in Japan indicated a significant improvement in the mRS grade between baseline and outcome within 1 year, regardless of the surgical technique, and shunt intervention was found to be effective 9).


Clinically, patients presenting with early or severe dementia have worse outcome, while those with a primary gait disturbance feature have better results 10) 11).


Clinical improvement of patients with iNPH can be sustained for 5-7 years in some patients, even if shunt revision surgery is needed multiple times. With earlier diagnosis and treatment and the increasing lifespan of the ageing population, the need for long-term follow-up after shunt surgery may be greater than it was in the past. Monitoring, identification and treatment of shunt obstruction is a key management principle 12).

Gait and Incontinence

Postoperative improvement of Gait and Urinary incontinence is obtained at an early stage 13) 14). In contrast, Dementia tends to improve gradually from after the third postoperative month. The family satisfaction increases as the symptom of Dementia improve. The satisfaction of the medical personnel tends to remain high after the first postoperative month 15).

Independent predictors

Independent predictors of improvement are the presence of gait impairment as the dominant symptom and shorter duration of symptoms 16).

see Caregiver burden in idiopathic normal pressure hydrocephalus.


1)

Kimura T, Yamada S, Sugimura T, Seki T, Miyano M, Fukuda S, Takeuchi M, Miyata S, Tucker A, Fujita T, Hashizume A, Izumi N, Kawasaki K, Nakagaki A, Sako K. Preoperative Predictive Factors of Short-Term Outcome in Idiopathic Normal Pressure Hydrocephalus (iNPH). World Neurosurg. 2021 Apr 22:S1878-8750(21)00596-9. doi: 10.1016/j.wneu.2021.04.055. Epub ahead of print. PMID: 33895373.
2)

Subramanian HE, Mahajan A, Sommaruga S, Falcone GJ, Kahle KT, Matouk CC. The subjective experience of patients undergoing shunt surgery for idiopathic normal pressure hydrocephalus. World Neurosurg. 2018 Jul 4. pii: S1878-8750(18)31425-6. doi: 10.1016/j.wneu.2018.06.209. [Epub ahead of print] PubMed PMID: 29981467.
3)

Hebb AO, Cusimano MD: Idiopathic normal pressure hydrocephalus: a systematic review of diagnosis and outcome. Neurosurgery 49:1166–1186, 2001
4) , 16)

McGirt MJ, Woodworth G, Coon AL, Thomas G, Williams MA, Rigamonti D. Diagnosis, treatment, and analysis of long-term outcomes in idiopathic normal-pressure hydrocephalus. Neurosurgery. 2005 Oct;57(4):699-705; discussion 699-705. PubMed PMID: 16239882.
5)

Meier U, Kiefer M, Neumann U, Lemcke J: On the optimal opening pressure of hydrostatic valves in cases of idiopathic normal-pressure hydrocephalus: a prospective randomized study with 123 patients. Acta Neurochir Suppl 96:358–363, 2006
6)

Wikkelsø C, Hellström P, Klinge PM, Tans JT: The European iNPH Multicentre Study on the predictive values of resistance to CSF outflow and the CSF Tap Test in patients with idiopathic normal pressure hydrocephalus. J Neurol Neurosurg Psychiatry 84:562–568, 2013
7)

Klinge P, Marmarou A, Bergsneider M, Relkin N, Black PM. Outcome of shunting in idiopathic normal-pressure hydrocephalus and the value of outcome assessment in shunted patients. Neurosurgery. 2005 Sep;57(3 Suppl):S40-52; discussion ii-v. Review. PubMed PMID: 16160428.
8)

Toma AK, Papadopoulos MC, Stapleton S, Kitchen ND, Watkins LD. Systematic review of the outcome of shunt surgery in idiopathic normal-pressure hydrocephalus. Acta Neurochir (Wien). 2013 Oct;155(10):1977-80. doi: 10.1007/s00701-013-1835-5. Epub 2013 Aug 23. Review. PubMed PMID: 23975646.
9)

Nakajima M, Miyajima M, Ogino I, Akiba C, Kawamura K, Kurosawa M, Kuriyama N, Watanabe Y, Fukushima W, Mori E, Kato T, Sugano H, Karagiozov K, Arai H. Shunt Intervention for Possible Idiopathic Normal Pressure Hydrocephalus Improves Patient Outcomes: A Nationwide Hospital-Based Survey in Japan. Front Neurol. 2018 Jun 7;9:421. doi: 10.3389/fneur.2018.00421. eCollection 2018. PubMed PMID: 29942280; PubMed Central PMCID: PMC6004916.
10)

Bugalho P, Alves L, Ribeiro O. Normal pressure hydrocephalus: a qualitative study on outcome. Arq Neuropsiquiatr. 2013 Nov;71(11):890-5. doi: 10.1590/0004-282×20130173. PubMed PMID: 24394877.
11)

Poca MA, Mataró M, Matarín M, Arikan F, Junqué C, Sahuquillo J. Good outcome in patients with normal-pressure hydrocephalus and factors indicating poor prognosis. J Neurosurg. 2005 Sep;103(3):455-63. PubMed PMID: 16235677.
12)

Pujari S, Kharkar S, Metellus P, Shuck J, Williams MA, Rigamonti D. Normal pressure hydrocephalus: long-term outcome after shunt surgery. J Neurol Neurosurg Psychiatry. 2008 Nov;79(11):1282-6. doi: 10.1136/jnnp.2007.123620. Epub 2008 Mar 20. PubMed PMID: 18356257.
13)

Savolainen S, Hurskainen H, Paljärvi L, Alafuzoff I, Vapalahti M: Five-year outcome of normal pressure hydrocephalus with or without a shunt: predictive value of the clinical signs, neuropsychological evaluation and infusion test. Acta Neurochir (Wien) 144:515–523, 2002
14) , 15)

Takeuchi T, Goto H, Izaki K, Tamura S, Sasanuma Z, Maeno K, Kikuchi Y, Tomii M, Koizumi Z, Watanabe Z, Numazawa S, Ito Y, Ohara H, Kowada M, Watanabe K. Postoperative patterns of improvement of symptoms and degrees of satisfaction in families of patients after operations for definite idiopathic normal pressure hydrocephalus: a long-term follow-up study]. No Shinkei Geka. 2007 Aug;35(8):773-9. Japanese. PubMed PMID: 17695775.

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.
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