Interests

In addition the Ances Laboratory is interested in dementias including Alzheimer’s Disease and Rapidly Progressive Dementias such as Cruetzfeld Jacob Disease. This group is interested in discovering early neuroimaging biomarkers of these diseases.

Differential Diagnosis of Rapidly Progressive Dementia

Neurodegenerative

  • Alzheimer’s disease
  • Frontotemporal Dementia
  • Dementia with Lewy bodies
  • Progressive Supranuclear Palsy
  • Vascular (Infarcts, amyloid angiopathy, Binswanger’s)

Autoimmune

  • Hashimoto’s encephalopathy (SREAT)
  • Sarcoid
  • Paraneoplastic/Antibody mediated encephalitis (VGKC, Yo, Hu, Ma, GAD65, CV2, NMDA)
  • Lupus cerebritis
  • Vasculitis
  • Behcet’s
  • Celiac Sprue

Infectious

  • HIV
  • Syphilis
  • Whipple’s disease
  • Lyme disease
  • Cryptococcus neoformans (other fungal)
  • Mycobacterium CNS infection
  • Viral encephalitis (including arbovirus)
  • Progressive Multifocal Leukoencephalopathy (JC Virus)
  • Subacute Sclerosing Panencephalitis
  • Systemic infections in older adults (UTI, pneumonia etc.)

Malignancies

  • Primary CNS Lymphoma (PCNSL)
  • Intravascular lymphoma
  • Gliomatosis Ccerebri

Toxic-Metobolic

  • Vitamin B12 deficiency
  • Thiamine deficiency
  • Porphyria
  • Heavy metal intoxication
  • Extrapontine myelinolysis
  • Drug abuse (inhalants)
  • Bismuth encephalopathy
  • Wilson’s
  • Hepatic or Uremic Encephalopathy
  • Mitochondrial

Other

  • Psychiatric
  • Obstructive Sleep Apnea (particularly in the elderly population)

Work-up of Rapidly Progressive Dementia

1st line investigation

  • Blood Tests – CBC, CMP, Mg, Phosphorus, LFT, ammonia, RPR, ANA, ESR, CRP, TSH, Vitamin B12, HIV, Paraneoplastic Ab panel, anti-thyroglobulin Ab, anti-thyroperoxidade Ab, heavy metal screen, bismuth level, ceruloplasmin (Wilson’s)
    CSF – Cell count and differential, arbovirus PCR, protein, glucose, 14-3-3 protein, tau, IgG synthesis rate and OCBs (Trotter studies), Mycobacterial culture, Fungal culture with cryptococcal antigen
  • Urine – urinalysis, 24 hour for heavy metals and copper (Wilson’s)
  • EEG – looking for periodic sharp wave complexes (PSWC)
  • Imaging – Brain MRI with and without contrast with DTI on a 1.5T scanner (scanner C). Call charge tech ahead of time and specify that you want the above.

2nd line investigation

  • Sleep study – contact Gabriela de Bruin, Victoria Sharma, Bob Bucelli, Enrique Alvarez, Erica Patrick, or Beth Ward to arrange this.
  • Blood Tests – Lyme Ab, copper and ceruloplasmin, ENA, ANCA, folic acid, B12 deficiency panel if applicable, blood smear
  • CSF – VDRL, flow cytometry and cytology, routine cultures and additional viral PCRs, paraneoplastic panel
  • Imaging – Consider CT chest, abdomen, pelvis to look for malignancy
  • Biopsy – especially in circumstances where the above results are non-diagnostic
  • Autopsy – contact path ahead of time