2024 ICD-10-CM Diagnosis Code A81.82

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Gerstmann-Sträussler-Scheinker syndrome

  • A81.82 is a billable/specific ICD-10-CM code that can be used to indicate a diagnosis for reimbursement purposes.
  • Short description: Gerstmann-Straussler-Scheinker syndrome
  • The 2024 edition of ICD-10-CM A81.82 became effective on October 1, 2023.
  • This is the American ICD-10-CM version of A81.82 – other international versions of ICD-10 A81.82 may differ.

Applicable To

  • GSS syndrome

The following code(s) above A81.82 contain annotation back-references

 that may be applicable to A81.82:

  • A00-B99  Certain infectious and parasitic diseases
  • A80-A89  Viral and prion infections of the central nervous system
  • A81  Atypical virus infections of central nervous system

Approximate Synonyms

  • Gerstmann-straussler-scheinker syndrome

Clinical Information

  • A very rare and fatal disorder of spongiform encephalopathy usually caused by mutations of the prion protein (prnp) gene. It is characterized by the accumulation of amyloid in the brain. Signs and symptoms include lack of motor coordination, unsteady gait, and difficulty walking. As the disease progresses, patients develop speech difficulties and dementia.
  • An autosomal dominant familial prion disease with a wide spectrum of clinical presentations including ataxia, spastic paraparesis, extrapyramidal signs, and dementia. Clinical onset is in the third to sixth decade of life and the mean duration of illness prior to death is five years. Several kindreds with variable clinical and pathologic features have been described. Pathologic features include cerebral prion protein amyloidosis, and spongiform or neurofibrillary degeneration. (from brain pathol 1998 jul;8(3):499-513; brain pathol 1995 jan;5(1):61-75)

ICD-10-CM A81.82 is grouped within Diagnostic Related Group(s) (MS-DRG v41.0):

  • 056 Degenerative nervous system disorders with mcc
  • 057 Degenerative nervous system disorders without mcc
  • 974 Hiv with major related condition with mcc
  • 975 Hiv with major related condition with cc
  • 976 Hiv with major related condition without cc/mcc

Convert A81.82 to ICD-9-CMCode History

  • 2016 (effective 10/1/2015): New code (first year of non-draft ICD-10-CM)
  • 2017 (effective 10/1/2016): No change
  • 2018 (effective 10/1/2017): No change
  • 2019 (effective 10/1/2018): No change
  • 2020 (effective 10/1/2019): No change
  • 2021 (effective 10/1/2020): No change
  • 2022 (effective 10/1/2021): No change
  • 2023 (effective 10/1/2022): No change
  • 2024 (effective 10/1/2023): No change

Diagnosis Index entries containing back-references to A81.82:

  • Gerstmann-Sträussler-Scheinker syndrome A81.82 (GSS)
  • Syndrome – see also Disease
    • Gerstmann-Sträussler-Scheinker A81.82 (GSS)