Show your best 3

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Show your best 3

• Karl Clebak

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Case Presentation

• 75 year old with rt shoulder numbness, lest sided
  trapezius muscle soreness fasciculation in left
  biceps. No headaches, dysphagia, dysphonia.

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Read

• There are low lying cerebellar tonsils, with
  protrusion approximately 11 mm below the
  basion-opisthion line. The posterior fossa is
  small. There is a large syrinx in the visualized
  portion of the cervical spine extending from the
  mid body of C2 inferiorly. The syrinx is
  incompletely imaged on this study. These
  findings are consistent with Chiari I
  malformation.

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Chiari Malformation

• Type I - fourth ventricle above foramen magnum,
  upper part of cervical cord displaced caudally,
  seen in pediatrics
• Type II - most common cerebellar vermis (
  cerebellar tonsils), medulla fourth ventricle
  herniated into upper cervical canal described
  here
• Type III - cerebellar vermis, medulla fourth
  ventricle protrude exteriorly as occipital
  encephalocele
• types III IV - progressive caudal displacement
  of cerebellar vermis, pons medulla below
  foramen magnum

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• Age affected
• 40-60 years
• symptoms in adolescence or adult life apparent
  at birth for types II and III
• Associated Symptoms
• syringomyelia, syringobulbia, deformities of
  vertebrae, cranial nerve palsies, hydrocephalus
  and hydromyelia associated with lumbosacral
  meningomyelocele obstructive sleep apnea
• related to loss of pharyngeal sensation

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Presentation

• Chief Concern
• cough-induced headache and neck pain, nausea,
  vomiting occasionally transient hydrocephalus,
  unsteadiness of gait, dysarthria, dysphagia,
  syncope (compromised medullary function)
• History of Present Illness
• pain at cranial-cervical junction aggravated by
  head movement or Valsalva
• General Physical
• rapidly increasing head circumference, lethargy,
  irritability

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More Physical

• HEENT
• bulging fontanelles
• scalp vein dilatation
• ocular findings
• decreased upward gaze
• Parinaud's syndrome - light-near dissociation
• light reflex may be disrupted in midbrain
  pretectal region without damage to more ventral
  near reflex fibers anatomic lesion is pretectal
  internuncial neurons serving reflex pupil
  constriction to light
• Parinaud's syndrome is usually caused by
  pinealomas or other dorsal midbrain lesions
• pupils are relatively large, often slightly
  unequal convergence-retraction nystagmus on
  attempted upgaze constriction to light is absent
  or very weak, but response to near stimulus is
  normal
• papilledema
• occasionally downbeat nystagmus accentuated on
  lateral gaze
• Neuro
• well compensated, progressive ataxia, peripheral
  neuropathy (tethered cord)

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Surgery

• Surgery
• shunt to direct ventricular fluid
• most commonly ventriculoperitoneal shunt
• absorptive surface of peritoneum may be
  inadequate in very small infants -
  ventriculoatrial shunt
• CSF may need to be shunted to pleural space

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Bonus Case

• Hip Pain

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References

• Arnold-Chiari malformation. Dynamed. Updated 2007
  Jul 05 0225 PM. Accessed 31 March 2008.