Introduction: Progressive supranuclear palsy (PSP) is an atypical parkinsonism characterized by early postural instability and falls, vertical supranuclear gaze impairment, pseudobulbar palsy, and frontal subcortical dysfunction, with little or no response to L-dopa. We describe a patient with a subacute onset of a PSP-like syndrome after liver transplantation (LT) and review the literature on extrapyramidal complications following organ transplantations. A wide spectrum of neurological manifestations after LT are indeed a significant source of morbidity and mortality, often occurring within 30 days from transplantation. However, the occurrence of an atypical parkinsonism has never been reported before. Case presentation: A 74-year-old man was referred because of a slowly progressive atypical rigid-akinetic syndrome which had started approximately one month after uncomplicated orthotropic LT. Cognitive problems were evident involving memory and frontal executive functions; some signs of apraxia, micrographia and mildly depressed mood were also observed. Additionally, neurogenic bladder dysfunction was detected. Brain magnetic resonance imaging showed midbrain atrophy without significant involvement of the pons, leading to the appearance of the typical “penguin” or “hummingbird” sign. Taken together, clinical and imaging features were consistent with the diagnosis of PSP. Symptoms were not modified by the subsequent anti-parkinsonian drug therapy. Conclusion: We hypothesize that, in this patient, a hypoxic insult of basal ganglia during surgery might have induced the onset of PSP because movement disorders frequently follow brain hypoxia and a particular vulnerability to hypoxia is present in specific brain regions, such as basal ganglia and neocortex. However, alternative hypotheses cannot be ruled out, such as a “trigger effect” of the surgical procedure for a neurodegenerative disease, neurologic consequences of chronic infections (hepatitis C or B), or a cyclosporine-induced parkinsonism. A diagnosis of PSP should be considered when features suggestive of an atypical extrapiramidal disease occur acutely or gradually after an organ transplantation.

Unusual presentation of atypical akinetic-rigid syndrome after liver transplantation: A case report and review of the literature

Lanza G;PENNISI, Giovanni;BELLA, Rita;
2015

Abstract

Introduction: Progressive supranuclear palsy (PSP) is an atypical parkinsonism characterized by early postural instability and falls, vertical supranuclear gaze impairment, pseudobulbar palsy, and frontal subcortical dysfunction, with little or no response to L-dopa. We describe a patient with a subacute onset of a PSP-like syndrome after liver transplantation (LT) and review the literature on extrapyramidal complications following organ transplantations. A wide spectrum of neurological manifestations after LT are indeed a significant source of morbidity and mortality, often occurring within 30 days from transplantation. However, the occurrence of an atypical parkinsonism has never been reported before. Case presentation: A 74-year-old man was referred because of a slowly progressive atypical rigid-akinetic syndrome which had started approximately one month after uncomplicated orthotropic LT. Cognitive problems were evident involving memory and frontal executive functions; some signs of apraxia, micrographia and mildly depressed mood were also observed. Additionally, neurogenic bladder dysfunction was detected. Brain magnetic resonance imaging showed midbrain atrophy without significant involvement of the pons, leading to the appearance of the typical “penguin” or “hummingbird” sign. Taken together, clinical and imaging features were consistent with the diagnosis of PSP. Symptoms were not modified by the subsequent anti-parkinsonian drug therapy. Conclusion: We hypothesize that, in this patient, a hypoxic insult of basal ganglia during surgery might have induced the onset of PSP because movement disorders frequently follow brain hypoxia and a particular vulnerability to hypoxia is present in specific brain regions, such as basal ganglia and neocortex. However, alternative hypotheses cannot be ruled out, such as a “trigger effect” of the surgical procedure for a neurodegenerative disease, neurologic consequences of chronic infections (hepatitis C or B), or a cyclosporine-induced parkinsonism. A diagnosis of PSP should be considered when features suggestive of an atypical extrapiramidal disease occur acutely or gradually after an organ transplantation.
File in questo prodotto:
File Dimensione Formato  
PSP after transplantation.pdf

accesso aperto

Descrizione: Articolo principale
Tipologia: Versione Editoriale (PDF)
Dimensione 2.69 MB
Formato Adobe PDF
2.69 MB Adobe PDF Visualizza/Apri

I documenti in IRIS sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.

Utilizza questo identificativo per citare o creare un link a questo documento: http://hdl.handle.net/20.500.11769/19232
Citazioni
  • ???jsp.display-item.citation.pmc??? ND
  • Scopus 2
  • ???jsp.display-item.citation.isi??? 2
social impact