Program Operations Manual System (POMS)
TN 30 (08-20)
COMPASSIONATE ALLOWANCES INFORMATION
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LISSENCEPHALY
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ALTERNATE NAMES
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Lissencephaly Type I; LIS1; Classical Lissencephaly; X-Linked Lissencephaly; XLIS;
Lissencephaly with Agenesis of the Corpus Callosum; Lissencephaly with Cerebellar
Hypoplasia; Microlissencephaly; Miller-Dieker Syndrome
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DESCRIPTION
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Lissencephaly is a brain malformation in which the physical structure of the brain did not develop
correctly during fetal development. Lissencephaly is characterized by the absence
of normal folds and ridges (convolutions) in the cerebral cortex, resulting in a nearly
smooth brain and an abnormally small head (microcephaly).
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DIAGNOSTIC TESTING, PHYSICAL FINDINGS, AND ICD-9-CM/ICD-10-CM
CODING
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Diagnostic testing: The diagnosis of lissencephaly is usually made by:
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Computed tomography (CT), or
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Magnetic resonance imaging (MRI).
Physical findings: Physical findings for lissencephaly may include:
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Hypo- (loss of muscle tone) or hypertonia (increased muscle tone);
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Difficulty controlling muscles (ataxia);
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Stiffness or spasticity of arms and legs;
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ICD-9: 742.2
ICD-10: Q04.3
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PROGRESSION
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The prognosis for children with lissencephaly is poor with many dying in infancy,
and the remainder showing no significant development beyond a 3 -5 month level.
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TREATMENT
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There is no way to reverse the effects of lissencephaly. Supportive management of
lissencephaly includes treatment of seizures, and physical and occupational therapies
to lessen spasticity. Feeding difficulties are treated with a gastrostomy tube. Respiratory
problems are the most common causes of death.
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SUGGESTED
PROGRAMMATIC ASSESSMENT* |
Suggested MER for Evaluation:
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Clinical history and examination that describes the diagnostic features of the impairment;
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Cranial MRI or CT scans; and
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Developmental assessment or psychological testing to address allegations of mental
impairment may be warranted.
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Suggested Listings for
Evaluation: |
DETERMINATION
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LISTING
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REMARKS
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Meets |
110.08
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111.02
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112.02
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Equals |
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* Adjudicators may, at their discretion, use the Medical Evidence of Record or the
listings suggested to evaluate the claim. However, the decision to allow or deny the
claim rests with the adjudicator.
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