1/29. Hypersomnia in Wilson's disease: an unusual symptom in an unusual case.Wilson's disease (WD) shows a wide heterogeneity in symptoms. In this case report we present hypersomnia as a symptom of WD. The male patient's complaints as fatigue, decreased level of concentration, and highly increased demand of sleeping started at his age of 21 years. No abnormality was found at physical examination. A moderate elevation in liver function tests was found, but all the other laboratory findings were within the normal range. The marked hypersomnia was verified by 24-h cassette EEG polisomnographic monitoring. No abnormality was found at physical examination. EEG, brain CT and MRI were normal. Neither toxic nor infectious disease was detectable. The diagnosis of WD was based on decreased coeruloplasmin level, increased baseline and forced urinary excretion of copper, and decreased level of serum copper. Kayser-Fleischer ring was not detectable. D-penicillamine (DPA) was introduced. At 8-10 months after the initiation of the therapy the patient's complaints gradually resolved. The control sleep record 14 months after the initiation of the DPA therapy was normal. Five years later the patient is currently on penicillamine treatment and he is free of any symptom.- - - - - - - - - - ranking = 1keywords = brain (Clic here for more details about this article) |
2/29. Nucleus of the tractus solitarius metastasis: relationship to respiratory arrest?BACKGROUND: A 52-year-old woman with metastases in brain and bone had clinical and radiological response to therapy but died about 10 weeks after diagnosis. General autopsy failed to identify a primary neoplasm or an anatomic cause of death. Investigation of sudden respiratory cessation was a consideration when undertaking an anatomic study of the brain. methods: review of patient records and careful examination of the brain following autopsy were carried out. RESULTS: The patient had terminal episodes of hypersomnia but episodes of sleep apnea were not observed. She received no respiratory support and no respiratory difficulties were recorded until she was pronounced dead at 7 a.m. autopsy revealed metastatic adenocarcinoma in a pattern suggestive of a primary pulmonary neoplasm, including multiple cerebral metastases, although no significant pulmonary lesions of any type were found. A 0.2 cm metastatic adenocarcinoma was found in the nucleus of the tractus solitarius (NTS). No other tumor was present in the brain stem. CONCLUSIONS: Unilateral destruction of the NTS in the medulla would have severely disturbed the most critical point of convergence of autonomic and voluntary respiratory control and of cardiocirculatory reflexes in the central autonomic network. It is postulated that this caused respiratory arrest during a state transition from sleeping to waking. Few metastases to the medulla are reported, most are relatively large, and several have caused respiratory symptoms before death. The very small metastasis in our patient could be the direct anatomic cause of death, and as such it is an unusual complication of metastatic disease of which clinicians should be aware. It is speculated that dysfunction of direct NTS connections to the pons or of connections passing close to the metastatic deposit resulted in terminal hypersomnia.- - - - - - - - - - ranking = 4keywords = brain (Clic here for more details about this article) |
3/29. A case of kleine-levin syndrome examined with SPECT and neuropsychological testing.A case of kleine-levin syndrome with typical periodic hypersomnia and bulimia was diagnosed. On examination with single photo emission tomography (SPECT) (CERETEC) during a relapse period and 2 weeks later there was marked cortical hypoperfusion of the frontal and temporal lobes, especially on the left side as well as in the right parietal lobe. Neuropsychological testing performed 1 week after a relapse showed a reduction in encoding to memory function of verbal learning indicating neocortical damage of the left fronto-temporal region. A follow-up 2 months later after the patient had spontaneously recovered showed only a slight left fronto-temporal disturbance. CT and MRI of the brain were normal although the MRI showed a large and asymmetric mamillary body. Neuropsychological testing 6 years after recovery showed pronounced reduction in short-time verbal and visual memory. Seven years after recovery SPECT demonstrated a normalized frontal perfusion but still a slight hypoperfusion in the left temporal lobe. Our results correlate to autopsy findings in two cases described previously.- - - - - - - - - - ranking = 1keywords = brain (Clic here for more details about this article) |
4/29. methylphenidate hydrochloride for excessive daytime sleepiness in a patient with myotonic dystrophy.A 28-year-old man with a history of myotonic dystrophy (MyD) is described. His progressively worsening excessive daytime sleepiness (EDS) was treated with methylphenidate hydrochloride. Polysomonography (PSG), the multiple sleep latency test (MSLT), middle latency-evoked response, and auditory event-related potentials were used to assess the cause of EDS. The PSG detected small numbers of central sleep apneic episodes. Mean sleep latency, which was determined by MSLT, increased from 5 min before treatment to 14 min after treatment. The Pa, P1, and N1 amplitudes at the Cz site increased after treatment, but P3 latency remained unchanged. These observations suggest that EDS and impairment of cognitive and information processing seen in a patient with MyD may be caused by a central nervous system disorder.- - - - - - - - - - ranking = 1.3787058704558keywords = central nervous system, nervous system (Clic here for more details about this article) |
5/29. A case of acute disseminated encephalomyelitis presenting hypersomnia with decreased hypocretin level in cerebrospinal fluid.A 12-year-old girl was diagnosed as having acute disseminated encephalomyelitis and manifested hypersomnia as the main clinical feature. magnetic resonance imaging (MRI) revealed lesions in the bilateral hypothalamus in addition to other multifocal brain lesions involving the cerebral white matter, brain stem, and basal ganglia. The level of hypocretin in cerebrospinal fluid was decreased in this patient. Corticosteroid treatment resulted in improvement of the hypersomnia and resolution of MRI lesions in the hypothalamus and other regions. This case suggests that the arousal state control mechanism related to the hypocretin peptide/receptor system may be impaired in some patients with acute disseminated encephalomyelitis.- - - - - - - - - - ranking = 2keywords = brain (Clic here for more details about this article) |
6/29. sleep attacks in Parkinson's disease induced by Entacapone, a COMT-inhibitor.We present a patient who suffered from sleep attacks after starting entacapone in addition to levodopa. Entacapone, a catechol-O-methyl transferase inhibitor, alters the pharmacokinetics of levodopa, leading to increase of levodopa concentration in plasma and brain. This mechanism is suspected to be involved in the pathophysiology of sleep attacks in this case.- - - - - - - - - - ranking = 1keywords = brain (Clic here for more details about this article) |
7/29. Systemic side effects of ophthalmic drops.Topical application of ocular drugs may cause serious adverse ocular or systemic side effects. Children are at greater risk for systemic side effects because ocular dosing is not weight-adjusted, and infants are especially vulnerable as a consequence of the inability to efficiently metabolize the drug, and/or an immature blood-brain barrier. Four pediatric patients are reported in whom treatment with topical brimonidine, a selective alpha-2 adrenoceptor agonist, indicated for the treatment of glaucoma, produced somnolence. Anti-glaucoma eyedrops that are not approved for use in children are still used frequently in pediatrics. Therefore, physicians should be aware of possible systemic side effects of ophthalmic drops.- - - - - - - - - - ranking = 1keywords = brain (Clic here for more details about this article) |
8/29. Hypersomnolence and increased REM sleep with low cerebrospinal fluid hypocretin level in a patient after removal of craniopharyngioma.Here we report a hypersomnolent girl with extensive hypothalamic damage after removal of a craniopharyngioma. The presence of a short sleep latency, sleep onset REM periods during a multiple sleep latency test (MSLT) and negative HLA DQB1*0602 typing suggested a diagnosis of symptomatic narcolepsy. Low cerebrospinal fluid hypocretin-1 level indicated destruction of hypocretin-producing neurons in the hypothalamus. An increased amount of REM sleep and a lack of REM sleep cyclicity documented by all-night polysomnography were different findings from previous reports of hypocretin-deficient idiopathic symptomatic narcolepsy. A more global hypothalamic lesion demonstrated by brain magnetic resonance imaging (MRI) after surgery seemed to cause marked disinhibition of REM sleep as well as hypersomnolence in this patient.- - - - - - - - - - ranking = 1keywords = brain (Clic here for more details about this article) |
9/29. Hypersomnia caused by isolated angiitis of the CNS.A 42-year-old woman presented gradual development of hypersomnia and memory disturbance. T2-weighted and FLAIR MRI scans revealed a high intensity abnormality in the hypothalamus. Stereotactic brain biopsy specimen revealed prominent infiltration of the lymphocytes in the vessel walls. Since there was no evidence of systemic angiitis, the diagnosis of isolated angiitis of the CNS (IAC) was made. Polysomnographic study showed decreased REM sleep and increased stage I sleep. Orexin A in the CSF was decreased. Hypersomnia and a decrease of the CNS orexin A improved after corticosteroid therapy. Hypersomnia can be an initial symptom of IAC if it involves the hypothalamus.- - - - - - - - - - ranking = 1keywords = brain (Clic here for more details about this article) |
10/29. Effect of genetically caused excess of brain gamma-hydroxybutyric acid and GABA on sleep.BACKGROUND: Exogenous gamma-hydroxybutyrate (GHB) increases slow-wave sleep and reduces daytime sleepiness and cataplexy in patients with primary narcolepsy. OBJECTIVE: To examine nighttime sleep and daytime sleepiness in a 13-year-old girl homozygous for succinic semialdehyde dehydrogenase (SSADH) deficiency, a rare recessive metabolic disorder that disrupts the normal degradation of 4-aminobutyric acid (GABA), and leads to an accumulation of GHB and GABA within the brain. methods: sleep interview, nighttime polysomnography, Multiple sleep Latency Tests, and continuous 24-hour in-lab recordings in the patient; overnight polysomnography in her recessive mother and in a 13-year-old female control. RESULTS: During quiet wakefulness, background electroencephalographic activity was slow and composed of 7-Hz activity. sleep stage 3/4 was slightly increased (28.1% of total sleep period, norms 15%-28%), and the daytime mean sleep latency was short in the patient (3 minutes 42 seconds, norms > 8 minutes). Stage 2 spindles were infrequent in the child (0.18/minute, norms: 1.2-9.2/minute) and her mother (0.65/minute) but normal (4.6/minute) in the control. At the beginning of the second night, a tonic-clonic seizure occurred, followed by a dramatic increase in stage 3/4 sleep, that lasted 46.3 % of the total sleep period, double the normal value. The mother showed a reduced total sleep time and rapid eye movement sleep percentage. DISCUSSION: This suggests that a chronic excess of GABA and GHB induces subtle sleep abnormalities, whereas increased slow-wave sleep evoked by a sudden event (here an epileptic seizure) may be caused by a supplementary increase in GABA and GHB.- - - - - - - - - - ranking = 5keywords = brain (Clic here for more details about this article) |
| | Next -> |