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Tethered spinal cord syndrome is a neurological disorder caused by tissue attachments that

limit the movement of the spinal cord within the spinal column. Attachments may occur

congenitally at the base of the spinal cord (conus medullaris) or they may develop near the

site of an injury to the spinal cord. These attachments cause an abnormal stretching of the

spinal cord. The course of the disorder is progressive. In children, symptoms may include

lesions, hairy patches, dimples, or fatty tumors on the lower back; foot and spinaldeformities; weakness in the legs; low back pain; scoliosis; and incontinence. This type of

tethered spinal cord syndrome appears to be the result of improper growth of the neural tube

during fetal development, and is closely linked to spina bifida. Tethered spinal cord

syndrome may go undiagnosed until adulthood, when pain, sensory and motor problems, and

loss of bowel and bladder control emerge. This delayed presentation of symptoms is related

to the degree of strain placed on the spinal cord over time and may be exacerbated during

sports or pregnancy, or may be due to narrowing of the spinal column (stenosis) with age.

Tethering may also develop after spinal cord injury and scar tissue can block the flow of

fluids around the spinal cord. Fluid pressure may cause cysts to form in the spinal cord, a

condition called syringomyelia. This can lead to additional loss of movement, feeling or the

onset of pain or autonomic symptoms.

Is there any treatment?

MRI imaging is often used to evaluate individuals with these symptoms, and can be used to

diagnose the location of the tethering, lower than normal position of the conus medullaris, or

presence of a tumor or fatty mass (lipoma). In children, early surgery is recommended to

prevent further neurological deterioration. Regular follow-up is important: retethering may

occur in some individuals during periods of rapid growth and may be seen between five to

nine years of age. If surgery is not advisable, spinal cord nerve roots may be cut to relieve

pain. In adults, surgery to free (detether) the spinal cord can reduce the size and furtherdevelopment of cysts in the cord and may restore some function or alleviate other symptoms.

Other treatment is symptomatic and supportive.

What is the prognosis?

With treatment, individuals with tethered spinal cord syndrome have a normal life

expectancy. However, some neurological and motor impairments may not be fully

correctable. Surgery soon after symptoms emerge appears to improve chances for recovery

and can prevent further functional decline.

What research is being done?

The NINDS conducts and supports research on disorders of the spinal cord. The goals of this

research are to find ways to prevent, treat, and cure these disorders.



Sindrom sumsum tulang belakang ditambatkan adalah gangguan neurologis yang disebabkan oleh

lampiran jaringan yang membatasi pergerakan dari sumsum tulang belakang dalam kolom tulang

belakang . Lampiran dapat terjadi kongenital di dasar sumsum tulang belakang ( konus medularis )

atau mereka dapat mengembangkan dekat lokasi cedera sumsum tulang belakang . Lampiran ini

menyebabkan abnormal peregangan dari sumsum tulang belakang . Jalannya gangguan yang

progresif . Pada anak-anak , gejala mungkin termasuk lesi , patch berbulu , lesung , atau tumor lemak

pada punggung bawah , kaki dan deformitas tulang belakang , kelemahan di kaki , nyeri pinggang ,

scoliosis , dan inkontinensia . Jenis sindrom sumsum tulang belakang ditambatkan tampaknya

merupakan hasil dari pertumbuhan yang tidak benar dari tabung saraf selama perkembangan janin ,

dan berhubungan erat dengan spina bifida . Sindrom sumsum tulang belakang ditambatkan mungkin

tidak terdiagnosis sampai dewasa , ketika rasa sakit , masalah sensorik dan motorik , dan hilangnya

kontrol usus dan kandung kemih muncul . Ini presentasi tertunda gejala berkaitan dengan tingkat

ketegangan ditempatkan pada sumsum tulang belakang dari waktu ke waktu dan dapat diperburuk

selama olahraga atau kehamilan , atau mungkin karena penyempitan tulang belakang ( stenosis )

dengan usia . Tethering juga dapat berkembang setelah cedera tulang belakang dan jaringan parutbisa menghalangi aliran cairan di sekitar sumsum tulang belakang . Tekanan fluida dapat

menyebabkan kista terbentuk di sumsum tulang belakang , suatu kondisi yang disebut syringomyelia

. Hal ini dapat mengakibatkan kerugian tambahan gerakan , perasaan atau timbulnya rasa sakit atau

gejala otonom.

Apakah ada pengobatan ?

Pencitraan MRI sering digunakan untuk mengevaluasi individu dengan gejala ini , dan dapat

digunakan untuk mendiagnosa lokasi tethering , lebih rendah dari posisi normal dari konus medularis

, atau adanya tumor atau massa lemak ( lipoma ) . Pada anak-anak , bedah awal dianjurkan untuk

mencegah kerusakan neurologis lanjut . Regular tindak lanjut adalah penting : retethering dapat

terjadi pada beberapa individu selama periode pertumbuhan yang cepat dan dapat dilihat antara 5-9

tahun . Jika operasi ini tidak dianjurkan , akar saraf sumsum tulang belakang dapat dipotong untuk

menghilangkan rasa sakit . Pada orang dewasa , operasi untuk gratis ( detether ) sumsum tulang

belakang dapat mengurangi ukuran dan pengembangan lebih lanjut dari kista di kabel dan dapat

mengembalikan beberapa fungsi atau meringankan gejala lainnya . Pengobatan lainnya adalah

simtomatik dan suportif .

Apa prognosis ?

Dengan pengobatan , individu dengan sindrom sumsum tulang belakang ditambatkan memiliki

harapan hidup normal. Namun, beberapa gangguan neurologis dan motorik mungkin tidak

sepenuhnya diperbaiki . Pembedahan segera setelah gejala muncul tampaknya meningkatkan

peluang untuk pemulihan dan dapat mencegah penurunan lebih lanjut fungsional .

Penelitian apa yang sedang dilakukan ?

The NINDS melakukan dan mendukung penelitian tentang gangguan sumsum tulang belakang .

Tujuan dari penelitian ini adalah untuk menemukan cara untuk mencegah , mengobati , dan

menyembuhkan gangguan ini .



Introduction

When there is disturbed or incomplete closure of the neural tube during embryonic life itleads to a developmental congenital defect called spina bifida (SB). Some of the vertebrae of

the spinal cord are not completely formed and remain open to produce a defect in the

vertebral column that may or may not be covered by the skin. The most common site of this

defect is the lumber or the sacral segment of the spinal cord. The incidence of SB is about

one in every 1000 births1,2.

Disorders associated with spina bifida

There are various disorders which are similar to SB or which may be associated with SB.These disorders include:

Anencephaly

Encephalocele

Tethered Spinal Cord

Chiari Malformation

Meckel syndrome

Dandy-Walker-Malformation

1. Anencephaly

This disorder refers to the absence of a large portion of the brain or the skull. It is also termed

as aprosencephaly or with an open cranium. It is the neural defect that occurs when the

superior portion of neural the tube fails to close. The possible risk factors for this abnormality

include low folic acid level in pregnant mothers and exposure to certain toxins or drugs

during pregnancy. Its incidence is one in 10,000 births3.

Clinical features of anencephaly include:

Absence of the skull or a part with exposure of brain tissue

http://www.spinabifida.net/4-issues-with-neural-tube-defects.html



http://www.spinabifida.net/Encephalocele


http://www.spinabifida.net/4-facts-on-tethered-cord-syndrome.html


http://www.spinabifida.net/cobalamin-vitamin-b12-folic-acid-deficiency-in-spina-bifida-neural-tube-defects.html



http://www.spinabifida.net/causes/prescription-drugs.html


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Absence of the brain or a portion

Facial abnormalities

Valvular defects of the heart3

Diagnosis: The following tests can be carried out to diagnose this defect in a pregnant

mother:

Abdomenial ultrasonography

Amniocentesis

Alpha-fetoprotein level

Test of pre-pregnancy serum folate level

Urine estriol level3

Treatment: There is no current therapy except genetic counseling.

Prognosis: The anomaly usually results in death within a few days of birth.

Prevention: Adequate folic acid intake may prevent this malformation from developing3.

2. Encephalocele

This is another of the neural tube defects which result from the incomplete closure of superior

part of the neural tube. Its other name is cranium bifidum. It sticks out of the skull throughopenings in the form of protrusions of brain tissue with its covering membranes. It has many

similarities with spina bifida. It is a rare anomaly that occurs at a rate of one in 5000 live

births4,5.

Types: If the protruded part of encephalocele contains only CSF along with membranes, it

may be termed as meningocele and if the brain tissue also protrudes with it, it is called

encephalomeningocele. Encephaloceles include the following types4:

Naso-frontal

Naso-ethmoidal

Naso-orbital

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Risk factors: Recent studies show an association of these congenital anomalies with the

following risk factors:

Exposure to cancer causing agents

Exposure to tryptan blue- a stain used to color dead tissues

Decreased level of folic acids5

Clinical manifestations: Patients with this malformation can have following features:

Hydrocephalus

Weakness of lower limbs

An abnormally small sized head

Disturbed muscle movement

Developmental delay

Vision problems

Mental and growth retardation

Seizures5

Meningitis, both pre-operative and post operative

Hydrocephalus

Blindness6

Complications: These include:

Management: The following investigations are conducted with pregnant women to diagnose

this anomaly:

Ultrasonography of the abdomen

MRI AFP-level

5,6

Reparative surgery is the treatment of choice at an early stage. Other treatment options are

supportive therapy, counseling, physical therapy, psychotherapy etc5, 6.

http://www.spinabifida.net/treatment/fetal-surgery.html



http://www.spinabifida.net/5-facts-about-hydrocephalus.html


http://www.spinabifida.net/complications/social/how-spina-bifida-affects-childhood.html








3. Tethered Cord Syndrome

This syndrome is characterized by pulling of the spinal cord at the base of the spinal canal

along with consequent clinical manifestations. It has many different forms like:

Dermiods

Dermal sinus tracts

Cystoceles

Tight filum terminale

Lipomenengiomyelocele

Split cord malformation7

.

Aetiology: Causes of TCSinclude:

Disturbed growth of the neural tube during fetal development

Spina bifida

Later spinal cord injury

Syringomyelia8

Clinical manifestations: Clinical manifestations of this anomaly include:

Skin lesions like hairy patches, dimples, or fatty tumors on the lower back

Paralysis of both lower limbs

Spine and feet deformities

Kyphoysis and scoliosis

Backache

Urinary incontinence

Urinary retention

Weakness of muscle and loss of sensations of touch, vibration, temperature, pain and

proprioception Urinary frequency, urgency, feeling of incomplete urination

http://www.spinabifida.net/malformations.html



http://www.spinabifida.net/complications.html



http://www.spinabifida.net/10-ways-to-manage-spina-bifida-back-pain.html



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· Occipital encephalocele along with micrecephaly

· Cleft palate

· Polycystic kidney disease

· Polydactyly

· Ambiguous genitals5

6. Dandy-Walker-Malformation

It is characterized by enlargement of the 4th ventricle along with a cyst that extends into the posterior cranial fossa. This malformation is also associated with neural tube defects5.

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References

1. Davies RW. Congenital vertebral anomalies: aetiology and relationship to spina bifida

cystica. J Med Genet 1975;12(3):280-8.

2. Moris G. Adult Complications of Spina Bifida Occulta [internet]. [last updated 2011

March 25; cited 2013 Aug 23]. Available from:

http://www.livestrong.com/article/244091-adult-complications-of-spina-bifida-occulta/.

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http://www.spinabifida.net/wp-content/uploads/2013/09/220px-Dandy-Walker-Variante_-_MRT_T2_sagittal.jpg




Anencephaly [internet]. [last updated 2011 May 1; cited 2013 Aug 23]. Available from:

http://www.ncbi.nlm.nih.gov/pubmedhealth/PMH0002547/.

3. Khan AN, Turnbull I, MacDonald S. Encephalocele imaging: review [Internet]. 2011

[lasted updated 2011 May 25; cited 2013 Aug 17]. Available from:

http://emedicine.medscape.com/article/403308-overview.

4. Anthony D, John G, Adam R, Jeffrey V, Geoffrey L. Frontoethmoidal encephaloceles:reconstruction and refinements. J Caraniofac Surg 2011;12(1):6-18.

5. What are Encephaloceles? [Internet]. [Cited 2013 Aug 17]. Available from:

http://www.bandbacktogether.com/encephalocele-resources/.

6. Yamada S, Lonser RR. Adult tethered cord syndrome. J spinal Disord 2000;13(4):319-23.

7. Agarwalla PK, Dunn IF, Scott RM, Smith ER. Tethered cord syndrome. Neurosurg Clin N

Am 2007;18(3):531-47.

8. Pang D, Wilberger JE. Tethered cord syndrome in adults. J Neurosurg 1982;57(1):32-47.

9. Ratliff J, Mahoney PS, Kline DG. Tethered cord syndrome in adults. South Med J

1999;92(12):1199-203.

http://www.ncbi.nlm.nih.gov/pubmedhealth/PMH0002547



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