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.

[Detection of active varicella-zoster virus (VZV) infection in patients with neurological complications]. / Prukaz aktivní infekce virem varicella zoster (VZV) u pacientu s neurologickými komplikacemi.

OBJECTIVES: When introduced into routine virological diagnosis of nervous system infections, PCR detection of viral DNA revealed the varicella-zoster virus (VZV) in cerebrospinal fluid (CSF) at much higher rates than expected. The aim of the study was to evaluate the frequency of VZV DNA detection in CSF of patients with neurological symptoms in correlation with their VZV-specific serological findings and clinical symptoms. MATERIAL AND METHODS: A total of 438 patients followed up in the neurology departments of the Motol and Královské Vinohrady University Hospitals and the Department of Infectious Diseases of the Bulovka University Hospital were screened for the presence of VZV-specific antibodies in serum and intrathecal antibodies in CSF. A home-brew nested PCR assay was used for detection of VZV DNA in CSF. Positive results were correlated with clinical findings. RESULTS: Intrathecal antibodies against VZV were detected in 19.6 % of the studied patients, VZV-specific IgM antibodies were present in serum of 17.3 % of the patients and VZV DNA was recorded in CSF of 9.4 % of the patients. The clinical diagnosis was confirmed in 16 patients positive for VZV DNA in CSF: encephalitis as a complication of neonatal varicella in a 2-week child; encephalitis or meningoencephalitis in 5 adult patients of whom three had a history of herpes zoster, one suffered from severe haemorrhagic focal encephalitis with fatal complications and one had encephalitis and myelitis; neuropathies in 4 patients, two with inflammatory polyneuropathy of unknown origin and two with brachial plexopathy, in one case preceded by herpes zoster; epileptic symptoms in 2 patients; multiple sclerosis in 3 patients and nonspecific symptoms of chronic fatigue in one patient. CONCLUSIONS: 1) PCR proved to be a suitable method for diagnosing VZV-mediated nervous system infections. 2) VZV DNA can be present in CSF of patients with a wide range of neurological symptoms, even with no history of either herpes zoster or varicella. 3) VZV DNA detection in CSF needs to be interpreted with caution and in correlation with case histories, clinical findings and electrophysiological and imaging data, especially in patients with chronic inflammatory disease receiving immunosuppressive therapy.

[Importance of urgent cerebrospinal fluid examination for early diagnosis of central nervous system infections]. / Význam urgentního vysetrení mozkomísního moku pro vcasnou diagnostiku neuroinfekcí.

To a certain extent, the cerebrospinal fluid (CSF) composition reflects the current status of the central nervous system (CNS). Therefore, studying changes in even the most essential CSF parameters provides enormous scope for obtaining valuable information about processes in the CNS in relation to its disorders. The article aims at presenting our current conception of urgent CSF examination with special emphasis on early diagnosis of central nervous infections. In particular, the focus is on evaluating energy conditions in the CSF compartment and permeability of the blood-brain and blood-CSF barriers, CSF cytology, detecting CNS tissue destruction and bleeding into CNS pathways and monitoring the levels of systemic inflammatory activity.

Mastoiditis complicated with Gradenigo syndrome and a hypertrophic pachymeningitis with consequent communicating hydrocephalus.

We present the clinical, radiological and pathological features of a case of a cranial hypertrophic pachymeningitis that developed in the course of mastoiditis and petrous apex inflammation and responded to immunosuppressive therapy only. Documented by the development of clinical findings, magnetic resonance imaging, cerebrospinal fluid changes, histopathology findings, by otosurgical intervention and finally by the insertion of a ventriculo-peritoneal shunt, the case illustrates a gradual development of pachymeningitis with consequent hydrocephalus and intracranial hypertension. We consider this disease development an example of immune-induced proliferative fibrotic changes in meninges.