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.
Subject(s)
Adult , Humans , Male , Autism Spectrum Disorder , Autistic Disorder , Creatine Kinase , Diagnosis , Encephalomyelitis , Intellectual Disability , Myoclonus , Neuroleptic Malignant Syndrome , Seizures , Status EpilepticusABSTRACT
Progressive supranuclear palsy (PSP) with predominant cerebellar ataxia (PSP-C) is a rare phenotype of PSP. The clinical and radiological features of this disorder remain poorly characterized. Through a retrospective case series, we aim to characterize the clinical and radiological features of PSP-C. Four patients with PSP-C were identified: patients who presented with prominent cerebellar dysfunction that disappeared with the progression of the disease. Supranuclear gaze palsy occurred at a mean of 2.0 ± 2.3 years after the onset of ataxia. Mild cerebellar volume loss and midbrain atrophy were detected on brain imaging, which are supportive of a diagnosis of PSP. Videos are presented illustrating the co-existence of cerebellar signs and supranuclear gaze palsy and the disappearance of cerebellar signs with disease progression. Better recognition and the development of validated diagnostic criteria would aid in the antemortem recognition of this rare condition.
Subject(s)
Humans , Ataxia , Atrophy , Cerebellar Ataxia , Cerebellar Diseases , Diagnosis , Disease Progression , Mesencephalon , Neuroimaging , Paralysis , Phenotype , Retrospective Studies , Supranuclear Palsy, ProgressiveABSTRACT
AIM: To study the anti-ulcer actions of the clinic experience prescription Shuangpu Powder and to provide experimental basis for further application. METHODS: Four kinds of experimental rodent ulcer models including aqueous extract excitability ulcer in rats,pyloric ligational ulcer in rats,hydrochloric acid-alcohol induced ulcer in rats and indomethacin induced ulcer in mice were used to observe its actions of anti-ulcer of Shuangpu Powder. RESULTS: The results showed that the Shuangpu Powder significantly decreased ulcer formation induced by stress,pylorus ligation,hydrochloric acid-alcohol and indomethacin. CONCLUSION: Shuangpu Powder has obvious anti-ulcer actions.