An interesting case of subacute sclerosing panencephalitis presenting with Balint's syndrome and dysautonomia

Swati Parida1, Nikhil Pandey2, Anand Kumar2

  • 1Department of Neurology, Kalinga Institute of Medical Sciences, Bhubaneswar, Odisha, India.

PubMed

Insights

Subacute sclerosing panencephalitis (SSPE), a rare measles virus infection, typically presents with neurological decline. This case highlights an unusual SSPE presentation with Balint's syndrome and autonomic dysfunction, emphasizing the need for high diagnostic suspicion.

Area of Science:

  • Neuroscience
  • Virology
  • Neuropathology

Background:

  • Subacute sclerosing panencephalitis (SSPE) is a rare, fatal neurodegenerative disease resulting from persistent measles virus infection.
  • SSPE typically manifests with progressive cognitive decline, behavioral changes, seizures, and motor deficits, ultimately leading to a vegetative state.

Observation:

  • This report details an atypical SSPE case in a 22-year-old male initially presenting with Balint's syndrome.
  • The patient subsequently developed myoclonus, cognitive decline, and autonomic dysfunction six months after symptom onset.
  • Autonomic dysfunction in SSPE is linked to central autonomic pathway involvement and can predict cardiac complications.

Findings:

  • Cerebrospinal fluid analysis and electroencephalography confirmed SSPE diagnosis.
  • The patient experienced rapid symptom progression and ultimately succumbed to the illness.
  • This case underscores the rare occurrence of Balint's syndrome as an initial SSPE manifestation.

Implications:

  • Atypical presentations of SSPE, including Balint's syndrome and autonomic dysfunction, require a high index of clinical suspicion.
  • Early diagnosis and intervention, though challenging, are crucial for managing SSPE.
  • Understanding rare SSPE presentations improves diagnostic accuracy and patient outcomes.

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