myotonic dystrophy/headache

We reported a 39-year-old man with myotonic dystrophy. He suffered from morning headache. Respiratory function tests showed restrictive pattern and arterial gas analysis showed hypoxia and hypercapnia with respiratory acidosis (PaCO2 50.8 mmHg, PaO2 63.8mmHg, pH 7.317, SaO2 89.8%). Polysomnograph

Results of the first comparative study using a Russian validated version of the Medical Outcomes Study Short Form-36 (MOS SF-36) are presented. Two hundreds and seventy-four patients with several neurological disorders - primary cephalgias (124 patients), strokes (120) and myotonic dystrophy (30)

BACKGROUND It is well known that myotonic dystrophy type 1 (DM1)--Curschmann-Steinert disease--is associated with white matter lesions in the brain. Further, DM1 patients may suffer from cardiac involvement and cardioembolic strokes. We report on the unique case of an adult-onset DM1 without cardiac

OBJECTIVE To report new disease components in a unique myotonic dystrophy type 1 (DM1) family previously described by us in which all affected members also had a sensorimotor neuropathy that co-segregated with markers flanking the DM1 locus. METHODS Clinical observations, electrophysiology,

OBJECTIVE To evaluate the potential of modafinil in reducing excessive daytime somnolence (EDS) and enhancing indexes of quality of life and mood in patients with myotonic dystrophy (DM). METHODS Forty patients with DM were randomized to receive modafinil and placebo for 14 days each, using a

BACKGROUND We examined the effects of pain site and intensity on function in patients with myotonic dystrophy type 1 (DM1) and facioscapulohumeral muscular dystrophy (FSHD) and chronic pain. METHODS Questionnaires assessing pain sites, pain extent (number of sites), pain intensity, and pain

We evaluated findings in 14 patients with myotonic dystrophy (MD) using magnetic resonance imaging of the brain and compared them with those in age-matched controls with headache. There was an increased incidence of ventriculomegaly and a lumpy and/or thick pattern of periventricular hyperintensity

Neurologic disorders may present or masquerade as pediatric sleep problems and fool the pediatrician, which may delay diagnosis and treatment. Many of the sleep problems in children with neurologic disorders arise directly from primary dysfunction or delayed maturation of their sleep-wake regulation

In neuromuscular diseases, respiratory disorder is related to sleep disorder. In Duchenne muscular dystrophy, respiratory muscle disorder progresses and induces alveolar hypoventilation. Hypoxemia and hypercapnia develop, requiring appropriate management. Hypoxemia first appears during sleep,

Pediatric neurologic diseases are often associated with different kinds of sleep disruption (mainly insomnia, less frequently hypersomnia or parasomnias). Due to the key-role of sleep for development, the effort to ameliorate sleep patterns in these children could have important prognostic benefits.

Patients with neuromuscular disease may suffer from nocturnal respiratory failure despite normal daytime respiratory function. The physiological reduction in muscle tone during sleep may be life-threatening in a patient with impaired muscle strength. Nocturnal respiratory failure may occur in

The cyclic alternations of wakefulness and sleep competing for the domain of brain activity are controlled by neuronal systems contained in the core of the brainstem, hypothalamus, thalamus, and basal forebrain. This organization encompasses complex neuroanatomic, neurophysiologic, and neurochemical

The circadian rhythmicity of plasma PRL has been studied in some neurological diseases in which hypothalamic involvement or abnormalities of brain neurotransmitters has been postulated. 11 patients with Steinert's myotonic dystrophy, 7 with cluster headache and 10 with Huntington's chorea have been

Modafinil is a wake-promoting agent that is pharmacologically different from other stimulants. It has been investigated in healthy volunteers, and in individuals with clinical disorders associated with excessive sleepiness, fatigue, impaired cognition and other symptoms. This review examines the use

BACKGROUND Normal swallowing function is divided into oral, pharyngeal, and oesophageal phases. The anatomy and physiology of the oral cavity facilitates an oral preparatory phase of swallowing, in which food and liquid are pushed towards the pharynx by the tongue. During pharyngeal and oesophageal