This document describes a case study of a 29-year-old female patient presenting with decreased sensation in her right hand and forearm, as well as ulcers and eruptions. She has a history of Arnold-Chiari malformation type 1 associated with syringomyelia. Physical examination found loss of superficial sensation in the right hand and forearm with involvement of the lateral spinothalamic tract and posterior columns. Nerve conduction studies indicate syringomyelia affecting the right lateral spinothalamic tract and central autonomic pathways, with mild involvement of the anterior horn cells. The patient likely has Arnold-Chiari malformation type 1 associated with syringomyelia causing dissociative anesthesia in the right upper

1. Syringomyelia
Electrodiagnosis
Dr Mohamed ibrahim khalil
Resident of physical medicine
Rheumatology and rehabilitation ,
Alexandria University EGYPT
Reverie-forever@hotmail.com

2. A 29 year-old female patient , married housewife from
Abu – Hommos presented with:
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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.

3. 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 .
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The patient didn’t seek medical advice and received analgesics .
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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 .
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7. 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.
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She sought medical advice , X ray on the right hand was
done , Performed culture sensitivity ,and received
antibiotics with no improvement ,
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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 .

8. 
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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.

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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

11. Physical examination

18. 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 .

19. 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

38. 
A case of operated Arnold Chiari Malformation
(Type1) associated with Syringohydromyelia and
severe central autonomic dysfunction
most propably :

42. 
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Most common type
Least severe
Asymptomatic in childhood
associated with syringomyelia
( 25 % of casses)

44. 
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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

45. 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.
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1.
With Deeper tonsillar herniation :
Ataxia , nystagmus , Dysphagia and Syncope ,
Apnea

46. 
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.

49. 
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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
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exceedingly rare and generally incompatible with
life. It causes severe neurological defects and is
associated with an occipital encephalocele
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this is a very rare disease ,incompatible with life.
It is characterized by a lack of cerebellar
development.
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50. 
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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.

54. 
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The most common form with Chiari type 1 is the non
communicating canalicular
The most common site is between C5-T1

55. 
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 )
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57. 
The dissociative anaethesia takes the Distribution of
Short Jacket - Long Sleeve
Because the most common site of the syrnx is
C5-T1

58. 
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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

61. 
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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)

63. 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 .