Syringomyelia

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Syringomyelia

  1. 1. Syringomyelia & Syringobulbia Dr. Osman Sadig Bukhari
  2. 2. <ul><li>Fluid filled cavity (syrnx), usually ant. to the </li></ul><ul><li>central canal, usually in za cervical cord, </li></ul><ul><li>sometimes extending to za thoracic cord </li></ul><ul><li>( syringomyela) & into za brainstem </li></ul><ul><li>(syringobulbia) </li></ul><ul><li>Aetiology: </li></ul><ul><li>It is due to rise of pressure within za closed </li></ul><ul><li>ventricular system consequent of blockage of </li></ul><ul><li>exit foramina of za 4 th ventricle 2nry to: </li></ul><ul><li>- Arnold Chiari malformation ( congenital </li></ul><ul><li>herniation of cerebellar tonsils through the </li></ul><ul><li>foramen magnum) </li></ul><ul><li>- Basal arachnoiditis </li></ul><ul><li>- Spinal cord trauma </li></ul><ul><li>NB : hydrocephalus may be asociated wz syringomyelia </li></ul>
  3. 3. <ul><li>Pathology: </li></ul><ul><li>Syrinx gradually destroys: </li></ul><ul><li>1- decussating S/T tracts </li></ul><ul><li>2- ant. horn cells </li></ul><ul><li>3- lateral C/S tracts </li></ul><ul><li>4- sympathetic tracts </li></ul><ul><li>5- trigeminal, 1X, X, X1 & X11 cranial N nuclei </li></ul><ul><li>and vestibular system as syrinx extends to </li></ul><ul><li>the medulla. </li></ul>
  4. 4. <ul><li>Clinical features : </li></ul><ul><li>- insidious onset at za 3 rd or 4 th decade with </li></ul><ul><li>slow progression. </li></ul><ul><li>- cervical & shoulder pain </li></ul><ul><li>- dissociated sensory loss I za chest & ULs. </li></ul><ul><li>( Cape distribution) </li></ul><ul><li>- painless burns & ulcers in za hands ( trophic) </li></ul><ul><li>- Charcot joints In za ULs. </li></ul><ul><li>- wasting of small muscles of za hands & loss </li></ul><ul><li>of one or more reflexes in za Uls. </li></ul><ul><li>- spastic paraparesis as disease progresses </li></ul><ul><li>- Horners, ataxia, bulbar palsy & loss of pain & temp in za face. </li></ul>
  5. 5. <ul><li>- kypho scoilosis, pes cavus & spina bifida are </li></ul><ul><li>common associations. </li></ul><ul><li>Investigations: </li></ul><ul><li>-plain X ray to show anomalies around F magnu </li></ul><ul><li>- MRI </li></ul><ul><li>Management </li></ul><ul><li>- Surgical decompression of za F magnum or </li></ul><ul><li>syrinx may arrest za progression & neurological </li></ul><ul><li>deficit, but No curative TR. </li></ul><ul><li>- Supportive measures. </li></ul><ul><li>Course : - disease gradually progressive over several decades & sudden deter may occur spontaneously or following trauma. </li></ul>

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