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Ethylmalonic Encephalopathy

Ethylmalonic encephalopathy (EE) is a rare metabolic disorder characterized by psychomotor regression and generalized hypotonia, which progresses into spastic tetraparesis, dystonia and global neurological failure. MRI shows necrotic lesions in the basal ganglia and brainstem. The encephalopathy is typically accompanied by petechia and orthostatic acrocyanosis. Chronic diarrhea is also common. Most patients with EE have [...]

+ Mutation-specific testing   + Prenatal testing

Ethylmalonic encephalopathy (EE) is a rare metabolic disorder characterized by psychomotor regression and generalized hypotonia, which progresses into spastic tetraparesis, dystonia and global neurological failure. MRI shows necrotic lesions in the basal ganglia and brainstem. The encephalopathy is typically accompanied by petechia and orthostatic acrocyanosis. Chronic diarrhea is also common. Most patients with EE have been from the Mediterranean basin or Arabic peninsula.

+ Mutation-specific testing   + Prenatal testing

Tests Available

ETHE1 Gene Sequencing & Del/Dup

FORMS AND DOCUMENTS

TEST DETAILS

Genes:
ETHE1
Clinical Utility:
  • Confirmation of biochemical diagnosis
  • Carrier testing
  • Prenatal diagnosis in at risk pregnancies
Lab Method:
Capillary Sequencing, Exon Array CGH

ORDERING

Test Code:
558
Turnaround Time:
4-5 weeks
Preferred Specimen:
2-5 mL Blood - Lavender Top Tube

BILLING

CPT Codes:
81479x1, 88271x10, 88291x1
List Price:
$1,260.00
Billing Information:
View Billing Policy
ICD Codes:
  • 348.3: Encephalopathy, not elsewhere classified

REFERENCES

  1. Mineri et al., (2008) J Med Genet 45:473-478.
  2. Tiranti et al., (2006) J Med Genet 43:340-346.
  3. Tiranti et al., (2009) Nature Med 15:200-205.

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