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Spectrum of Movement Disorders of Late-Onset Niemann-Pick Disease Type C.

Jasmine PariharDeepa DashBhawana AggarwalMadhulika KabraRoopa RajanDeepti VibhaRajesh Kumar SinghRohit BhatiaNeerja GuptaManjari Tripathi
Published in: The Canadian journal of neurological sciences. Le journal canadien des sciences neurologiques (2021)
Niemann-Pick disease type C (NPC), is a rare lysosomal storage disorder, which has a variable presentation based on the age of onset. We describe five adult/adolescent-onset NPC cases presenting with a range of movement disorders along with vertical supranuclear gaze palsy as part of the clinical presentation. A diagnostic delay of 4-17 years from the symptom onset was found in this case series. A high index of clinical suspicion in adult/adolescent patients presenting with vertical supranuclear gaze palsy along with various movement disorder phenomenology can help in the early diagnosis of NPC.
Keyphrases
  • late onset
  • young adults
  • childhood cancer
  • early onset
  • mental health
  • case report