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Dr Mohamed ibrahim khalil Resident of physical medicine Rheumatology and rehabilitation , Alexandria University EGYPT
Syringomyelia Electrodiagnosis
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 .
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 .
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
Diagnosis: A case of operated Arnold Chiari Malformation
(Type1) associated with Syringohydromyelia and severe central autonomic dysfunction
most propably :
MORVAN SYNDROME
What is Arnold Chiarri malformation ?
Chiarri Type 1 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
The symptoms of Chiari malformation type I 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.
With Deeper tonsillar herniation : 1. 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.
Chiarri Type 2 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
Type 3 exceedingly rare and generally incompatible
with life. It causes severe neurological defects and is associated with an occipital encephalocele
Type 4 this is a very rare disease ,incompatible with
life. It is characterized by a lack of cerebellar
development.
Chiarri Type 3 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
Type 4 this is a very rare disease ,incompatible with
life. It is characterized by a lack of cerebellar
development.
What Is The Relation between chiarri Type I and syryngomyelia ?
The most common form with Chiari type 1 is the non communicating canalicular
The most common site is between C5-T1
Symptoms of syringomyelia ?
The First most common symptom is Dissociative anaethesia: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 )
Symptoms of syringomyelia ?
The dissociative anaethesia takes the Distribution of
Short Jacket - Long Sleeve
Because the most common site of the syrnx isC5-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
Affected spinal cord tracts and columns: 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)
Back to our case
Atypical case of syringomyelia doesn’t
show Definite Pyramidal signs of UMNL ?
Facilitated H / M Ratio is Related more to
Extrapyramidal Lesion 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 .
Is there a relation between Syryngomyelia and This skin changes ?
La Main succulante of syringomyelia(subcutaneous thickening and swollen fingers)
Morvan’s Syndrome Progressive Loss of Pain sensation , Ulceration ,Loss of soft tissues and Resorption of Phalanges )
