What are neurologic manifestations of Sturge-Weber syndrome (SWS)?

Updated: Dec 26, 2018
  • Author: Masanori Takeoka, MD; Chief Editor: George I Jallo, MD  more...
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Answer

In the brain, LAs demonstrated by structural neuroimaging may be unilateral or bilateral [9] ; unilateral angiomas are more common. Functional neuroimaging may demonstrate a greater area of involvement than structural neuroimaging. [10] This is called a structural-versus-functional mismatch. (See Workup.)

The neurological manifestations of SWS vary, depending on the location of the LAs (which most commonly are located in the parietal and occipital regions) and the secondary effects of the angiomas. These neurological morbidities include the following:

  • Seizures - May be intractable

  • Focal deficits - Including hemiparesis and hemianopsia, both of which may be transient (called "strokelike episodes")

  • Headaches

  • Developmental disorders - Including developmental delay, learning disorders, and mental retardation/intellectual disability; more common when angiomas are bilateral

Focal or generalized motor seizures usually begin in the first year of life. Profound seizure activity sometimes may be observed, with resultant further neurological and developmental deterioration. [11] Seizure control is thought to improve the neurological outcome, and epilepsy surgery may be beneficial for refractory seizures. Therefore, diagnosing and treating the disease early, before permanent damage to the brain occurs, is preferable. (See Prognosis and Treatment.)

Progressive, characteristic calcifications in the external layers of the cerebral cortex underlying the angiomatosis associated with ipsilateral cortical atrophy frequently develop and progress with age, occasionally extending anteriorly to the frontal and temporal lobes. (See Pathophysiology and Etiology.)

Certain CNS malformations have been associated with SWS; other neurocutaneous disorders are included in the differential diagnosis.


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