This is a sagital T2 weighted MRI of a 28 years old worker who came to the Neurology unit complaining of headache in the back of head and the nape associated  with paresthesia and weakness of the  hands and limbs.
On examination cerebellar signs were elicited but there was no significant muscle wasting.
 

What is the Diagnosis?


ANSWER

ARNOLD-CHIARI MALFORMATION TYPE 1


The Arnold-Chiari malformation is a congenital malformation of the base of the skull with varying degrees of herniation of cerebellum and medulla into the spinal canal.
Mainly three types
  • type I - cerebellar ectopia
  • type II
  • type III


Arnold-Chiari malformation TYPE 1 is the most common form.It is generally asymptomatic during childhood.


In symptomatic patients, it is associated with:
spinal diseases - 50% of cases: syringomyelia
cranial disorders - 10% of cases: obstructive hydrocephalus

CLINICAL FEATURES

Symptoms and Signs of Brain Stem,Cranial nerves and cerebellar compression
  • Gait ataxia
  • Spastic quadraparesis
  • Downbeat nystagmus or rotatory nystagmus
  • Bulbar symptoms
Occipital headaches - induced by coughing and straining


The blockage of CSF flow may also cause a syrinx to form, eventually leading to syringomyelia and eventually patient will develop symptoms of cord compression

INVESTIGATIONS

  1. MRI - the investigation of choice
  2. CT - best with contrast

TREATMENT

In symptomatic patients
  • Decompression surgery - removes the lamina of the first cervical vertebrae and part of the occipital bone of the skull to relieve pressure
  • Ventricular peritoneal shunting if there is evidence of hydrocephalus or raised intracranial pressure.

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9 comments

  1. Anonymous Says:
  2. sah

     
  3. Anonymous Says:
  4. Arnold Chiari Malformation

    Greetings from Greece!

     
  5. Anonymous Says:
  6. its arnold chiare syndrome

     
  7. Anonymous Says:
  8. Arnold-Chiari malformation
    dr.winzo

     
  9. Anonymous Says:
  10. arnold- chiari syndrome

     
  11. Anonymous Says:
  12. Yep, prolapse of vermis and medulla oblangata in foramen magnum .... Arnold chiari malformation.

     
  13. Anonymous Says:
  14. arnold chiari syndrome

     
  15. Anonymous Says:
  16. ARNOLD-CHIARI MALFORMATION.

    Thank you!!!! More cases are always welcomed!! Keep up the AWESOME work!

     
  17. AMSR Says:
  18. There is another form of paresthesia called asmr, autonomous sensory meridian response. The only difference is that the tingling is highly pleasurable.

     

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