J Mov Disord.  2017 May;10(2):99-101. 10.14802/jmd.16058.

Progressive Encephalomyelitis with Rigidity and Myoclonus in an Intellectually Disabled Patient Mimicking Neuroleptic Malignant Syndrome

• Xu Z
• Prasad K
• Yeo T

• KMID: 2379644
• DOI: http://doi.org/10.14802/jmd.16058

Abstract

We present a case of 32-year-old male with profound mental retardation and autism spectrum disorder who had presented with seizures, rigidity and elevated creatine kinase and was initially diagnosed as neuroleptic malignant syndrome (NMS). The patient subsequently had a complicated clinical course, developing refractory status epilepticus, which lead to the eventual diagnosis of progressive encephalomyelitis with rigidity and myoclonus (PERM). We discuss the clinical similarities and differences between NMS and PERM, and highlight the need to consider alternative diagnoses when the clinical picture of NMS is atypical, particularly in this patient group where the history and clinical examination may be challenging.

Keyword

Progressive encephalomyelitis with rigidity; neuroleptic malignant syndrome; autistic disorder

MeSH Terms

Adult
Autism Spectrum Disorder
Autistic Disorder
Creatine Kinase
Diagnosis
Encephalomyelitis*
Humans
Intellectual Disability
Male
Myoclonus*
Neuroleptic Malignant Syndrome*
Seizures
Status Epilepticus
Creatine Kinase