Syringomyelia ( Morvan syndrome)  Electrodiagnosis
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Syringomyelia ( Morvan syndrome) Electrodiagnosis

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Syringomyelia ( Morvan syndrome)  Electrodiagnosis Syringomyelia ( Morvan syndrome) Electrodiagnosis Presentation Transcript

  • Syringomyelia Electrodiagnosis Dr Mohamed ibrahim khalil Resident of physical medicine Rheumatology and rehabilitation , Alexandria University EGYPT Reverie-forever@hotmail.com
  • A 29 year-old female patient , married housewife from Abu – Hommos presented with:      Decreased sensation of the right hand and forearm. Painless ulcers and scattered eruption on the fingers and Palmer aspect of the right hand. Ambutated distal phalynx of the index finger. Deformed distal phalynx of the Little finger Swollen right hand and fingers.
  • The condition started 3 years ago with gradual onset of :  Numbness of the right upper limb , shoulder and right half of the face which gradually Progressed to  Decreased sensation of the right upper limb  Neck Pain  Drowsiness and intermittent frontal headache increased on straining  blurring of vision and tinnitus .  The patient didn’t seek medical advice and received analgesics .  After a year she was admitted to Neurosurgery department , MRI Brain was done ,Showed mild herniation of cerebellar tonsils through Foramen magnum She Performed Surgical Decompression operation at the base of the skull , The Headache improved , while Lost sensation of the right hand and forearm Persists .  View slide
  • After 8 months the Patient experienced :  swelling of the right hand  bluish discoloration under the nail  Painless small eruption at the tip of the right index finger which progressively ruptured leaving a deep ulcer with offensive smell at the tip of the right index finger.  She sought medical advice , X ray on the right hand was done , Performed culture sensitivity ,and received antibiotics with no improvement ,  Ten days later ,her index finger showed gangrene at the terminal phalanx , and surgical amputation of the distal phalanx of the right index was done . View slide
  •      2 weeks later , Many hypopegmented skin lesions and painless vesicular eruptions which scattered on her right palm and fingers and Progressively enlarged , ruptured , ulcerate and then scarred . Excessive sweating and abnormal hand Temperature changes ( sometimes very cold or very warm) Associated deformity of the Distal phalynx of the little finger . Recent complain of numbness of toes and planter aspect of both feet She sought Medical advice , and referred by a dermatologist to our department to do Nerve conduction study and to exclude Leprotic neuritis.
  •      Past Medical History : NO DM - NO HTN Family History : Irrelevant Vital signs : Pulse 95 b/min Respiration 16 /min Blood Pressure 125/90 Temperature 37 C No vesicular eruption on the left hand , Trunk , Lower Limbs No history of Dermatologic infection in the family
  • Physical examination
  • Sensory examination : a) Superficial : Fine touch : intact on both hands , But much more less on the right hand than the left . Pain and Temperature : ? Lost on the right hand and forearm Normal on the left hand . b) Deep: *Sense of movements and Position : Normal on both hands *Vibration sense : Intact on both hands . Much more less on the right hand than the left . Normal sensory examination of both lower limbs .
  • Motor examination of both upper limbs :  Normal Muscle state No wasting ( ?oedema of both hands ) ,  Normal Tone and Power of both upper limbs  ? Hyporeflexia of both upperlimbs . General Neurologic examination : Normal Reflexes : ankle just elicited , Knee reflex 2+ Extensor Panter : unequivocal Musculoskeletal examination :  Normal Range of motion of all joints of upper limb  Deformity of distal phalynx of the Little finger  ?Thickned ulnar nerve at the elbow  Crepitus on both elbows  ? Plantar fascitis of both lower limbs
  •  A case of operated Arnold Chiari Malformation (Type1) associated with Syringohydromyelia and severe central autonomic dysfunction most propably :
  •      Most common type Least severe Asymptomatic in childhood associated with syringomyelia ( 25 % of casses)
  •   Diagnosis can be difficult because not all patients will have the classical sign of deeply herniated tonsils There may be herniation herniation on radiological examination without clinical significance
  • Symptoms is variabale and may include : 1. Headaches. ( Duration of headache min- few hours) 2. Nausea but not vomiting . 3. Visual symptoms. 4. Numbness and Pain in upper limbs 5. Neck , upperlimb , facial Pain 6. Ttinnitus 7. Muscle weakness.   1. With Deeper tonsillar herniation : Ataxia , nystagmus , Dysphagia and Syncope , Apnea
  •  Untreated Chiari malformation type 1 may also result in the development of fluid filled cavities in the brain (hydrocephalus) or spinal cord (syrinx) which may eventually lead to syringomyelia. This can lead to irreversible damage to the brain or spinal cord.
  •     less common than type 1 more severe than type I. it is usually accompanied by a lumbar myelomenigocele which results to either a partial or complete paralysis below the spinal defect. It becomes symptomatic in infancy or early childhoodis the least frequent form of Chiari malformation, characterized by a lack of growth of the cerebellar hemispheres  exceedingly rare and generally incompatible with life. It causes severe neurological defects and is associated with an occipital encephalocele  this is a very rare disease ,incompatible with life. It is characterized by a lack of cerebellar development. 
  •     is the least frequent form of Chiari malformation, characterized by a lack of growth of the cerebellar hemispheres exceedingly rare and generally incompatible with life. It causes severe neurological defects and is associated with an occipital encephalocele this is a very rare disease ,incompatible with life. It is characterized by a lack of cerebellar development.
  •   The most common form with Chiari type 1 is the non communicating canalicular The most common site is between C5-T1
  •  The First most common symptom is Lost Pain and tempreature sensation with Preservasion of Light touch Due to earlier involvement of the crossing Lateral spinothalamic tract before the Cunneate Tract ( nearer to central canal ) 
  •  The dissociative anaethesia takes the Distribution of Short Jacket - Long Sleeve Because the most common site of the syrnx is C5-T1
  •     With more Detorioration and enlargement of the syrinx , other columns and tracts are involved : AHCs with wasting in small hand muscles (before CST) Anterior column , corticospinal tracts ( UMNL) Posterior cord is affected in late stages
  •       Right Lateral spinothalamic tracts ( anterolateral column)  Loss of pain and temp sensation in the right UL Very mild involvement of AHC  ( Giant waves without axonopathy – No clinical weakness - ) Posterior column or ( Cuneate tracts )  SEP Amplitude Decreased for the right hand and both lower limbs ( more for right Cunneate tracts – Light touch is intact both sides but much more less on the right UL) Central Autonomic tracts ( Intermediolateral cell column in the Lateral Horn ) Abnormal SSR - Vasomotor changes in the Right Hand Extrapyramidal tracts UMNL ( H/M Ratio ) (Normal Gait , No Spasticity , No Sphincteric , No Clonus nor hyperreflexia) ???No definate evidence of Pyramidal tract affection ( or ?? very Mild Pyramidal ) (Normal Gait , No Spasticity , No Sphincteric , No Clonus nor hyperreflexia)
  • Pyramidal tracts Have Inhibitory effect on Muscle tone and reflexes Extrapyramidal Tract have Fascilitatory effect to muscle tone and reflexes : Reticulospinal tract,Vestibulospinal tract and Rubruspinal tract Normally there is a balance between Inhibitory and fascilitatory effect of both. If there is Selective Lesions in the Fascilitatory EPT  Inhibitory effect of the Pyramidal tracts will take the upper hand  HypoReflexia , Hypotonia , and Increased H/M ratio ( This explaines the Increased H/M ratio in Hypotonic CP) If there is a Selective Lesion in the Pyramidal tracts  Fascilitatory effect of the EPT takes the upper hand  Hypertonia , Hyperreflexia , Clonus , Extensor Planter .