Hypocretin Neurons In Lateral Hypothalamus Research Articles

Narcolepsy with cataplexy and parkinson’s disease – A case report

Narcolepsy with cataplexy (NC) is a sporadic neurological disease with prevalence 0.02–0.067% and it is caused by the loss of hypocretin neurons in lateral hypothalamus. NC is associated with HLA DQB1*06:02 allele. Parkinson‘s disease (PD) is a degenerative disorder manifested by hypokinetic-rigid syndrome, caused by degeneration of dopamine-producing cells in the substantia nigra pars compacta. The prevalence of PD is 0.1–0.2%. In addition to cardinal motor symptoms (akinesia, rigidity, tremor and postural instability), autonomic and sensory disturbances as well as cognitive, behavioral and sleep problems can occur during the disease progression. This is a case report presentation. An 86-year-old Caucasian woman suffering from NC was diagnosed at our department in 1980s. Daytime polysomnography (PSG) was performed, demonstrating typical SOREMPs. The patient was treated by phenmetrazin and imipramin or clomipramin. In 1995 the patient was retired and her narcoleptic symptoms became less prominent. The patient stopped the therapy and dropped out from the contact with our department. Hypokinetic-rigid syndrome with symmetric rigidity and akinesia in upper extremities and mild gait difficulty with instability was diagnosed when the patient was 83 year old. Patient’s symptoms stabilized after pramipexol 0.7 mg bid initiation. At the age of 85, the patient reported disturbing hypersomnia and frequent falls, which led to reevaluation in our department. Patient reported frequent daytime sleep attacks with variable duration. The sleepiness was present in rest as well as during social activities. The falls, mostly backwards were not caused by emotions nor associated with decreased muscle tone. Sometimes these were preceded by lightheadedness and pre-syncopal syndromes, but mostly were related to gait instability. Patient did not report hypnagogic hallucinations or sleep paralysis. Night PSG revealed frequent PLMS, severe sleep apnea (AHI 43), MSLT confirmed narcolepsy (short sleep latency and 2 SOREMPs), HLA typing showed haplotype HLA DQB1*06:02. Clinical examination showed typical parkinson’s syndrome (motor subscale of UPDRS 28) with good response to dopaminergic treatment and the diagnosis of idiopathic PD was established. We report this case because the co-occurrence of NC and PD is very rare according to the literature. IGA MZ ÈR: NT 13238–4/2012.