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Hypervalinemia and hyperleucine-isoleucinemia(HVLI)

MedGen UID:
1719306
Concept ID:
C5394277
Disease or Syndrome
Synonyms: BRANCHED-CHAIN AMINOTRANSFERASE DEFICIENCY; HVLI; Hypervalinemia or hyperleucine-isoleucinemia
 
Gene (location): BCAT2 (19q13.33)
 
Monarch Initiative: MONDO:0100058
OMIM®: 618850

Definition

Hypervalinemia and hyperleucine-isoleucinemia (HVLI) is a branched-chain amino acid metabolic disorder characterized by highly elevated plasma valine and leucine concentrations. The patient presented in adulthood with headache and mild memory impairment, and had abnormal symmetric white matter signals on brain MRI (Wang et al., 2015). [from OMIM]

Clinical features

From HPO
Headache
MedGen UID:
9149
Concept ID:
C0018681
Sign or Symptom
Cephalgia, or pain sensed in various parts of the head, not confined to the area of distribution of any nerve.
Hoffmann sign
MedGen UID:
78828
Concept ID:
C0277839
Sign or Symptom
A Hoffmann test is performed by flicking the fingernail of the long finger, from dorsal to volar, on each hand while the hand was supported by the examiner's hand. The test was done with the neck in the neutral position and then with the neck maximally forward flexed. Any flexion of the ipsilateral thumb and/or index finger was interpreted as a positive test.
Increased CSF protein concentration
MedGen UID:
329971
Concept ID:
C1806780
Finding
Increased concentration of protein in the cerebrospinal fluid.
Brisk reflexes
MedGen UID:
382164
Concept ID:
C2673700
Finding
Tendon reflexes that are noticeably more active than usual (conventionally denoted 3+ on clinical examination). Brisk reflexes may or may not indicate a neurological lesion. They are distinguished from hyperreflexia by the fact that hyerreflexia is characterized by hyperactive repeating (clonic) reflexes, which are considered to be always abnormal.
Elevated circulating alanine aminotransferase concentration
MedGen UID:
57740
Concept ID:
C0151905
Finding
An abnormally high concentration in the circulation of alanine aminotransferase (ALT).
Hypervalinemia
MedGen UID:
120652
Concept ID:
C0268573
Disease or Syndrome
Valinemia is an inborn error of metabolism characterized clinically by vomiting, feeding difficulties, hypotonia, and developmental delay, and biochemically by high concentrations of valine in serum and urine (Wada, 1965).
Hyperleucinemia
MedGen UID:
451032
Concept ID:
C0268576
Disease or Syndrome
An increased concentration of leucine in the blood.
Hyperisoleucinemia
MedGen UID:
869234
Concept ID:
C4023657
Disease or Syndrome
An increased concentration of isoleucine in the blood.

Recent clinical studies

Diagnosis

Wang XL, Li CJ, Xing Y, Yang YH, Jia JP
J Inherit Metab Dis 2015 Sep;38(5):855-61. Epub 2015 Feb 5 doi: 10.1007/s10545-015-9814-z. PMID: 25653144

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