A longitudinal study of an unusual rapidly progressive dementia case: A Case report

Overview

Case Presentation

A 74-year-old, right-handed male was admitted with complaints of jaw and bilateral hand movements for 3 months and rapidly progressive dementia for 1 month, coupled with intermittent visual hallucinations and raving.

Physical Examination

  • On admission, a neurologic examination indicated prominent cognitive impairment and psychiatric symptoms.
  • Particularly, he failed to complete the Mini-Mental State Examination (MMSE) and had a neuropsychiatric inventory (NPI) score of 50. 
  • Magnetic Resonance imaging indicated ischemia within the periventricular area and centrum semiovale. 
  • The haematological screening was unremarkable, including a routine blood test, tumour marker, biochemistry, folate, vitamin B12, and thyroid function.
  • A routine cerebrospinal fluid (CSF) test demonstrated normal pressure, leukocytosis, glucose, and chlorine but mildly elevated protein levels. 
  • A video electroencephalogram indicated increased slow activities without epileptic discharge.

Medical History

  • His past medical history was traced back to 5 years ago when he suffered from rectal carcinoma and received surgery and chemotherapy.

Clinical Examination

  • The patient exhibited multiple involuntary movements (jaw tremors, motor tremors of hands), his hands always groped about, and would fall backward when he was walking or seated. 
  • He could not recognize his family or location. 
  • The patient also experienced aberrant motor behaviour and night-time behaviour.

Treatment

  • Olanzapine 2.5 to 5 mg per night and was administered pramipexole and compound levodopa. 
  • The patient was also treated with immunotherapy, including intravenous immunoglobulin 0.4 g/kg per day for 5 days followed by intravenous methylprednisolone pulse therapy 500 mg per day for 5 days. 
  • Methylprednisolone was decreased to 80 mg per day and mycophenolate mofetil 500 mg was administered twice a day.

Clinical Outcome

  • On the 9th day of immunotherapy, the patient was cooperative for MMSE evaluation and an improvement in orientation, immediate recall and naming, and repetition function

Adapted from:

  1. Liu X, Fan Z, Chen X, Zhang Y, He F, Ma X, Ke Q. Case report: A longitudinal study of an unusual rapidly progressive dementia case. Front Neurol. 2023 Apr 6;14:1151130. doi: 10.3389/fneur.2023.1151130. PMID: 37090977; PMCID: PMC10118976