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Progressive dialysis encephalopathy

R J Lederman, C E Henry

    Annals of Neurology
    |September 1, 1978
    PubMed
    Summary
    This summary is machine-generated.

    Progressive dialysis encephalopathy, a fatal neurological syndrome, causes speech issues, dementia, and seizures despite hemodialysis. Aluminum toxicity is a suspected cause, but treatment remains ineffective.

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    Area of Science:

    • Neurology
    • Nephrology

    Background:

    • Progressive dialysis encephalopathy (PDE) is a recently recognized, fatal neurological syndrome.
    • Patients undergoing hemodialysis can develop severe neurological abnormalities despite seemingly successful treatment.

    Purpose of the Study:

    • To review the clinical, electroencephalographic (EEG), and neuropathological findings in patients with progressive dialysis encephalopathy.
    • To characterize the neurological manifestations and diagnostic features of this condition.

    Main Methods:

    • Review of clinical data from 42 patients diagnosed with progressive dialysis encephalopathy.
    • Summary of electroencephalographic (EEG) and neuropathological findings.

    Main Results:

    • The most characteristic early sign is sudden hesitant, nonfluent speech (predominantly dysphasic).

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  • Other common symptoms include myoclonus, dementia, seizures, and gait difficulty.
  • EEGs show significant abnormalities (high-voltage spike-wave patterns, slow activity) disproportionate to clinical severity; clinical and EEG findings are nearly diagnostic.
  • Conclusions:

    • The combination of specific clinical and EEG findings aids in diagnosing progressive dialysis encephalopathy.
    • Neuropathological changes are mild and nonspecific, with aluminum neurotoxicity being a leading hypothesis.
    • Current treatment options for progressive dialysis encephalopathy are unsatisfactory.