Rare Case of Late-Onset Narcolepsy Type 1.

A 69-year-old male developed symptoms typical of the diagnosis of narcolepsy type 1 without any previous triggering events. First, daytime sleepiness occurred, soon followed by cataplexy. Nocturnal polysomnography revealed rapid eye movement (REM) sleep behavior disorder, a apnea-hypopnea index of 25.8 events/h, and no sleep-onset REM. Multiple Sleep Latency Test showed a mean sleep latency of 2.1 min and REM sleep in 3 tests. HLA DQB1*06:02 was positive and hypocretin-1 in cerebrospinal fluid unmeasurable. A treatment with 50 mg clomipramine controlled the cataplexy; excessive daytime sleepiness was sufficiently managed by repeated naps. The administration of 0.25 mg of clonazepam subjectively improved REM sleep behavior disorder. Bilevel Positive Airway Pressure improved the apnea-hypopnea index without important influence on sleepiness. Our unique case demonstrates that even elderly subjects can develop narcolepsy type 1.

Relations of non-motor symptoms and dopamine transporter binding in REM sleep behavior disorder.

The aim of this study was to evaluate associations of motor and non-motor symptoms with dopamine transporter binding in prodromal stage of synucleinopathies. We examined 74 patients with idiopathic REM sleep behavior disorder (RBD), which is a prodromal synucleinopathy, and 39 controls using Movement Disorders Society-Unified Parkinson's Disease Rating Scale (MDS-UPDRS), Montreal Cognitive Assessment, University of Pennsylvania Smell Identification Test (UPSIT), Farnsworth-Munsell 100 hue test, orthostatic test, Scales for Outcomes in PD-Autonomic, Beck depression inventory-II, State-Trait Anxiety Inventory, and video-polysomnography. Electromyographic muscle activity during REM sleep was quantified according to Sleep Innsbruck-Barcelona criteria. In 65 patients, dopamine transporter single-photon emission computed tomography (DAT-SPECT) imaging was performed, putaminal binding ratio was calculated and scans were classified as normal, borderline, or abnormal. Compared to controls, RBD patients had significantly more severe scores in all examined tests. Patients with abnormal DAT-SPECT had higher MDS-UPDRS motor score (p = 0.006) and higher prevalence of orthostatic hypotension (p = 0.008). Putaminal binding ratio was positively associated with UPSIT score (p = 0.03) and negatively associated with tonic (p = 0.003) and phasic (p = 0.01) muscle activity during REM sleep. These associations likely reflect simultaneous advancement of underlying pathology in substantia nigra and susceptible brainstem and olfactory nuclei in prodromal synucleinopathy.

Smoking Prevalence and Its Clinical Correlations in Patients with Narcolepsy-cataplexy.

Narcolepsy-cataplexy (NC) is a chronic neurological disease with suggested autoimmune etiopathogenesis. Nicotine stimulates central nervous system and smoking increases the risk of autoimmune diseases. Assessment of smoking habits and its correlation to clinical parameters among 87 adult NC patients (38 male, 49 female) included night polysomnography and multiple sleep latency test. In our sample, 43.7% NC patients were regular smokers, and 19.5% former smokers compared to 22.2%, and 12.6%, respectively, in the general population. Patients started to smoke in the mean age of 20.0 (SD ±6.0) years. 72.2% of NC smokers started to smoke before the onset of NC and the mean of the delay between smoking onset and NC onset was 9.1 (±5.8) years. We found a direct correlation between smoking duration and the number of awakenings, duration of N1 sleep, REM sleep latency, and apnoea/hypopnoea index (AHI), and, on the contrary, indirect correlation between smoking duration and N3 sleep duration, showing that smoking duration consistently correlates with sleep macrostructure. Smoking is highly prevalent in NC and has relationship with clinical features of NC.

Narcolepsy with cataplexy in patients aged over 60 years: a case-control study.

OBJECTIVE: Narcolepsy with cataplexy (NC) is a chronic disabling disease; however, there are insufficient data on older NC subjects. METHODS: A cross-sectional evaluation on health and social status, including intensity and progression of NC symptoms, was performed on 42 NC patients (age 71.9 years ± 7.5) and 46 age-and-sex-matched controls (age 72.2 years ± 7.0). RESULTS: A greater proportion of patients than controls suffered from hypertension and type 2 diabetes. More controls had a history of treated depression; however, according to the Geriatric Depression Scale, more NC patients scored in the range of depression. There were no significant differences in Addenbrook Cognitive Examination scores. Average physical fitness assessed by the Short Physical Performance Battery was lower in the NC group. The frequency of meeting with family, friends, and participation in hobbies or sports did not differ between the two groups. CONCLUSIONS: Symptoms of NC were present throughout life. Comorbidities and lower physical fitness, which are known to be present in young and middle-aged NC subjects, were also present in older patients. Although NC subjects were less professionally active during their lifetime, they did not differ from controls in important social parameters in older age.

Narcolepsy with cataplexy and Parkinson's disease. Case Report.

We present the case of an 86 year old female, in whom narcolepsy with cataplexy (NC) manifested at 52 years of age. She was treated by an amphetamine-like drug phenmetrazine and tricyclic antidepressants for more than 10 years. Hypokinetic-rigid syndrome manifested at 83 years of age and Parkinson´s disease (PD) was diagnosed. Detailed examination at the age of 86 confirmed the previous diagnosis of NC and the diagnosis of PD. Severe periodic limb movements in sleep, severe sleep apnea, REM sleep behavior disorder and restless legs syndrome, which are frequently comorbid in NC and PD, were revealed. The patient's somnolence worsened, apparently accentuated by pramipexole treatment, as changing therapy to levodopa led to a reduction of sleepiness.