![]() ![]() Later, other case reports of more or less typical NC with onset after 50 years were published. In both cases, the diagnosis of NC was considerably delayed (15 years in the first case, misdiagnosed as secondary epilepsy). reported 2 cases: the first was a male with symptom onset at 72 years and the second a female at 84 years. The few previously reported cases of NC with an onset at an older age were initially misdiagnosed and, thus, the diagnosis was delayed. In the past, it was not rare that the diagnosis of narcolepsy was reached with a delay of 10 or more years. In our patient, the diagnosis of NT1 was reached 5 months after the first symptoms. However, NT1 subjects suffered from more frequent type 2 diabetes, arterial hypertension, and obesity and had lower physical fitness in old age. We did not find any major differences in social activities and quality of life in comparison to controls. These patients have been suffering from NT1 from a young age and have been treated for years. Our previous study looked into the health and quality of life in NT1 patients aged 60 years and older. Other studies did not find any difference in symptom severity according to age of onset but suggested a decline of cataplexy frequency and a milder course in older age in general. Some clinical and polysomnographic findings indicate that young age at onset is associated with increased severity of the condition, with a higher frequency of cataplexy and decreased mean sleep latency on MSLT. As for other symptom characteristics, we could not distinguish any differences from NT1 with younger age at onset. This is not very common in younger subjects, and we suppose that retirement might be the reason. DiscussionĪlthough MSLT and MWT confirmed severe EDS, the patient did not regard this as a problem. The last clinical control was done 2 years after the diagnosis and the treatment and clinical status of the patient were unchanged. He refused taking any stimulants and he was satisfied with scheduled naps after meals. However, sleepiness did not present a problem to the patient (other than the loss of his driving license). Within the MWT, the subject fell asleep in all 4 tests with a mean sleep latency of 0.25 min. Second nocturnal polysomnography performed with bilevel positive airway pressure showed sleep of a very poor quality, SOREM, sleep fragmentation, and multiple RBD manifestations. ![]() After a year on this treatment, a polysomnography and Maintenance of Wakefulness Test (MWT) were performed because the patient asked for a renewal of his driving license. To control RBD, the patient took 0.25 mg of clonazepam before a night’s sleep with a subjective improvement of RBD symptoms. Bilevel positive airway pressure in the automatic mode (expiratory pressure 4–16 mbar, pressure support 4–6 mbar) controlled obstructive sleep apnea (residual apnea-hypopnea index 1.6, T90 12%) with mild improvement of EDS. Based on the patient’s complaints, we raised the dosage to 50 mg of clomipramine and achieved a complete and permanent cataplexy resolution. Cataplexy disappeared for a few months when taking 25 mg but reoccurred occasionally afterwards. The first treatment was clomipramine because cataplexy was the patient’s major problem. Standard CSF analysis results were within the normal range.ĭiagnoses of NT1 with comorbid RBD and obstructive sleep apnea were set according to The International Classification of Sleep Disorders, Third Edition (ICSD-3, 2014). The patient’s serum and CSF was negative for IgLON5-IgG using commercially available cell-based assay (Euroimmun, Germany). A brain MRI did not reveal any abnormalities. ![]() The level of hypocretin in cerebrospinal fluid (CSF) was immeasurably low. ![]() Surprisingly, he scored 7 points on the Epworth Sleepiness Scale (ESS). The patient fell asleep in all 5 tests with a mean sleep latency of 2.1 min. On the following day, Multiple Sleep Latency Test (MSLT) confirmed EDS. Obstructive apneas were found with an apnea-hypopnea index of 25.8 events/h with low saturation (basal saturation 92%, 62% of time below 90% saturation ). No sleep-onset REM (SOREM) during the night was seen (REM sleep latency was 117 min). The nocturnal polysomnography showed sleep fragmentation (a sleep effectivity of 73%) and rapid eye movement (REM) sleep without atonia with behavioral manifestations (somniloquy, gesticulations, upper limb complex movements) corresponding to REM sleep behavior disorder (RBD). Objective neurological examination did not reveal any important neurological, physical, nor cognitive findings. ![]()
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