Fulminant encephalopathy with basal ganglia hyperintensities in HIV-infected drug users

Neurology. 2011 Mar 1;76(9):787-94. doi: 10.1212/WNL.0b013e31820e7b4e.

Abstract

Objective: To define a clinical syndrome associated with active drug abuse in HIV-infected individuals.

Methods: We performed a retrospective review to identify individuals treated at the Johns Hopkins Hospital from 1993 to 2008 who were HIV-infected and were actively abusing drugs and had bilateral basal ganglia lesions on MRI. They were identified using a key word search in the radiology database, autopsy database, and the Moore HIV clinic database. Clinical, laboratory, and radiographic findings were correlated to define the syndrome.

Results: Ten individuals were identified who presented with a change in mental status or seizures, used cocaine or cocaine with heroin, had uncontrolled HIV infection (>190,000 copies/mL of plasma), elevated CSF protein (63-313 mg/dL), and diffuse hyperintense bilateral basal ganglia lesions on imaging. The majority of patients (8/10) had renal failure and despite supportive therapy most (7/9) ultimately died (median survival 21 days). Postmortem examination in one individual showed the presence of overwhelming microglial activation in the basal ganglia. The 2 surviving individuals were started on combined antiretroviral therapy (CART) during hospitalization.

Conclusion: We describe a unique clinical syndrome of a fulminant encephalopathy associated with primarily basal ganglia involvement in HIV-infected drug abusers. This syndrome is a rare but serious condition that is associated with a high mortality rate. Early CART institution may be useful and neuroprotective in this disorder, although this requires further investigation.

Publication types

  • Case Reports
  • Comparative Study
  • Research Support, N.I.H., Extramural
  • Research Support, Non-U.S. Gov't

MeSH terms

  • AIDS Dementia Complex / complications
  • AIDS Dementia Complex / pathology*
  • Adult
  • Basal Ganglia / pathology*
  • Drug Users*
  • Female
  • HIV Infections / complications
  • HIV Infections / pathology*
  • Humans
  • Male
  • Middle Aged
  • Retrospective Studies
  • Substance-Related Disorders / complications
  • Substance-Related Disorders / pathology*
  • Syndrome