Syringomyelia

1. SyringomyeliaSyringomyelia
&&
SyringobulbiaSyringobulbia
Dr. Osman Sadig Dr. Osman Sadig
BukhariBukhari

2. Fluid filled cavity )syrnx(, usually ant. to theFluid filled cavity )syrnx(, usually ant. to the
,central canal, usually in za cervical cord,central canal, usually in za cervical cord
sometimes extending to za thoracic cordsometimes extending to za thoracic cord
syringomyela( & into za brainstem (syringomyela( & into za brainstem (
)syringobulbia()syringobulbia(
:Aetiology:Aetiology
It is due to rise of pressure within za closedIt is due to rise of pressure within za closed
ventricular system consequent of blockage ofventricular system consequent of blockage of
exit foramina of za 4exit foramina of za 4
thth
: ventricle 2nry to: ventricle 2nry to
Arnold Chiari malformation ) congenital -Arnold Chiari malformation ) congenital -
herniation of cerebellar tonsils through theherniation of cerebellar tonsils through the
)foramen magnum)foramen magnum
Basal arachnoiditis -Basal arachnoiditis -
Spinal cord trauma -Spinal cord trauma -
NB : hydrocephalus may be asociated wz syringomyelia NB : hydrocephalus may be asociated wz syringomyelia

3. :Pathology:Pathology
:Syrinx gradually destroys:Syrinx gradually destroys
decussating S/T tracts -1decussating S/T tracts -1
ant. horn cells -2ant. horn cells -2
lateral C/S tracts -3lateral C/S tracts -3
sympathetic tracts -4sympathetic tracts -4
trigeminal, 1X, X, X1 & X11 cranial N nuclei -5trigeminal, 1X, X, X1 & X11 cranial N nuclei -5
and vestibular system as syrinx extends toand vestibular system as syrinx extends to
.the medulla.the medulla

4. Clinical featuresClinical features::
insidious onset at za 3 -insidious onset at za 3 -
rdrd
or 4 or 4
thth
decade with decade with
.slow progression.slow progression
cervical & shoulder pain -cervical & shoulder pain -
.dissociated sensory loss I za chest & ULs -.dissociated sensory loss I za chest & ULs -
)Cape distribution ()Cape distribution (
)painless burns & ulcers in za hands ) trophic -)painless burns & ulcers in za hands ) trophic -
.Charcot joints In za ULs -.Charcot joints In za ULs -
wasting of small muscles of za hands & loss - wasting of small muscles of za hands & loss -
.of one or more reflexes in za Uls.of one or more reflexes in za Uls
spastic paraparesis as disease progresses -spastic paraparesis as disease progresses -
Horners, ataxia, bulbar palsy & loss of pain & - Horners, ataxia, bulbar palsy & loss of pain & -
. temp in za face . temp in za face

5. kypho scoilosis, pes cavus & spina bifida are -kypho scoilosis, pes cavus & spina bifida are -
.common associations.common associations
:Investigations:Investigations
plain X ray to show anomalies around F magnu-plain X ray to show anomalies around F magnu-
MRI -MRI -
ManagementManagement
Surgical decompression of za F magnum or -Surgical decompression of za F magnum or -
syrinx may arrest za progression & neurologicalsyrinx may arrest za progression & neurological
.deficit, but No curative TR.deficit, but No curative TR
.Supportive measures -.Supportive measures -
CourseCourse:: - disease gradually progressive over - disease gradually progressive over
several decades & sudden deter may occur several decades & sudden deter may occur
. spontaneously or following trauma . spontaneously or following trauma