Wolfram-like syndrome

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Wolfram-like syndrome
Autosomal dominant - en.svg
Synonyms Wolfram syndrome 2, DIDMOAD syndrome
Pronounce N/A
Specialty Endocrinology, Neurology, Genetics
Symptoms Diabetes mellitus, Optic atrophy, Deafness, Neurological disorders
Complications N/A
Onset Childhood or early adulthood
Duration Lifelong
Types N/A
Causes Mutations in the CISD2 gene
Risks Family history of the condition
Diagnosis Genetic testing, Clinical evaluation
Differential diagnosis Wolfram syndrome, Diabetes insipidus, Optic neuropathy
Prevention N/A
Treatment Symptomatic treatment, Insulin therapy, Hearing aids
Medication N/A
Prognosis Progressive, with variable severity
Frequency Rare
Deaths N/A


A rare genetic disorder with symptoms similar to Wolfram syndrome


Wolfram-like syndrome is a rare genetic disorder that shares several clinical features with Wolfram syndrome, but is caused by different genetic mutations. It is characterized by a combination of diabetes mellitus, optic atrophy, and other neurological and endocrine abnormalities.

Genetics[edit | edit source]

Wolfram-like syndrome is typically inherited in an autosomal dominant pattern, meaning that a single copy of the altered gene in each cell is sufficient to cause the disorder. This is in contrast to Wolfram syndrome, which is usually inherited in an autosomal recessive manner. The genetic mutations responsible for Wolfram-like syndrome are distinct from those causing Wolfram syndrome. While Wolfram syndrome is often associated with mutations in the WFS1 gene, Wolfram-like syndrome may involve mutations in other genes that have not been fully characterized.

Clinical Features[edit | edit source]

The clinical presentation of Wolfram-like syndrome can vary, but it typically includes:

Diagnosis[edit | edit source]

Diagnosis of Wolfram-like syndrome is based on clinical evaluation, family history, and genetic testing. Genetic testing can help differentiate it from Wolfram syndrome by identifying the specific mutations involved.

Management[edit | edit source]

There is currently no cure for Wolfram-like syndrome, and treatment is primarily supportive. Management strategies may include:

  • Diabetes Management: Insulin therapy and regular monitoring of blood glucose levels.
  • Vision Support: Regular ophthalmologic evaluations and supportive measures for vision loss.
  • Hearing Aids: For those with hearing impairment.
  • Neurological Care: Physical therapy and other interventions to manage neurological symptoms.

Prognosis[edit | edit source]

The prognosis for individuals with Wolfram-like syndrome varies depending on the severity of symptoms and the specific genetic mutations involved. Early diagnosis and management of symptoms can improve quality of life.

See also[edit | edit source]

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Contributors: Prab R. Tumpati, MD