Canellas Francesca, Lin Ling, Julià Maria Rosa, Clemente Antonio, Vives-Bauza Cristofol, Ollila Hanna M, Hong Seung Chul, Arboleya Susana M, Einen Mali A, Faraco Juliette, Fernandez-Vina Marcelo, Mignot Emmanuel
J Clin Sleep Med. 2014 Sep 15;10(9):1011-8. doi: 10.5664/jcsm.4040.
Cases of narcolepsy in association with psychotic features have been reported but never fully characterized. These patients present diagnostic and treatment challenges and may shed new light on immune associations in schizophrenia.
Our case series was gathered at two narcolepsy specialty centers over a 9-year period. A questionnaire was created to improve diagnosis of schizophrenia or another psychotic disorder in patients with narcolepsy. Pathophysiological investigations included full HLA Class I and II typing, testing for known systemic and intracellular/synaptic neuronal antibodies, recently described neuronal surface antibodies, and immunocytochemistry on brain sections to detect new antigens.
Ten cases were identified, one with schizoaffective disorder, one with delusional disorder, two with schizophreniform disorder, and 6 with schizophrenia. In all cases, narcolepsy manifested first in childhood or adolescence, followed by psychotic symptoms after a variable interval. These patients had auditory hallucinations, which was the most differentiating clinical feature in comparison to narcolepsy patients without psychosis. Narcolepsy therapy may have played a role in triggering psychotic symptoms but these did not reverse with changes in narcolepsy medications. Response to antipsychotic treatment was variable. Pathophysiological studies did not reveal any known autoantibodies or unusual brain immunostaining pattern. No strong HLA association outside of HLA DQB106:02 was found, although increased DRB303 and DPA1*02:01 was notable.
Narcolepsy can occur in association with schizophrenia, with significant diagnostic and therapeutic challenges. Dual cases maybe under diagnosed, as onset is unusually early, often in childhood. Narcolepsy and psychosis may share an autoimmune pathology; thus, further investigations in larger samples are warranted.
已有发作性睡病伴精神病性特征的病例报道,但从未得到充分描述。这些患者带来了诊断和治疗方面的挑战,可能为精神分裂症的免疫关联提供新线索。
我们的病例系列是在9年时间里于两个发作性睡病专科中心收集的。设计了一份问卷,以改善对发作性睡病患者中精神分裂症或其他精神病性障碍的诊断。病理生理学研究包括完整的HLA I类和II类分型、检测已知的系统性和细胞内/突触神经元抗体、最近描述的神经元表面抗体,以及对脑切片进行免疫细胞化学检测以发现新抗原。
共确定了10例病例,其中1例为精神分裂症伴情感障碍,1例为妄想性障碍,2例为精神分裂症样障碍,6例为精神分裂症。在所有病例中,发作性睡病均首先在儿童期或青春期出现,随后在一段可变的间隔时间后出现精神病性症状。这些患者有幻听,这是与无精神病的发作性睡病患者相比最具鉴别性的临床特征。发作性睡病治疗可能在触发精神病性症状中起了作用,但这些症状并未随着发作性睡病药物的改变而逆转。对抗精神病药物治疗的反应各不相同。病理生理学研究未发现任何已知的自身抗体或异常的脑免疫染色模式。除了HLA DQB106:02外,未发现强烈的HLA关联,尽管DRB303和DPA1*02:01增加值得注意。
发作性睡病可与精神分裂症同时发生,带来重大的诊断和治疗挑战。双重病例可能诊断不足,因为发病异常早,通常在儿童期。发作性睡病和精神病可能有共同的自身免疫病理;因此,有必要对更大样本进行进一步研究。