Raffaele M

Sleep features in Tourette's syndrome, neuroacanthocytosis and Huntington's chorea

Twenty-one patients affected by extrapyramidal disorders were polygraphically recorded during spontaneous nocturnal sleep for two consecutive nights to assess their sleep and movement patterns. The patients (pts) sample included: Gilles de La Tourette syndrome (TS, nine pts), neuroacanthocytosis (NA, six pts) and Hungtingtons chorea (HC, six pts). Sleep recording included C3/A2, 01/A2, ROC/LOC, submental EMG, EKG, nasal airflow thoracoabdominal respirogram, bilateral anterior tibialis and other EMGs, in relation to the individual distribution of the abnormal movements. According to our observations, abnormal movements always decreased but never ceased completely during sleep. Sleep efficiency (SE) was nearly always poor with a high percentage of wakefulness after sleep onset (WASO) and increased number of arousals. REM sleep was often reduced and in some cases (3 TS pts) incompletely defined as far as its microstructural aspects. Slow wave sleep (SWS) was reduced in HC, normal in NA, and increased in all TS patients with the exception of the two adult subjects more severely affected, while the percentage of stage 2 was not affected. Spindling was increased in NA, HC and in the two most severely affected adult TS patients.

Recurrent status epilepticus as the main feature of Hashimoto’s encephalopathy

Hashimotos encephalopathy (HE) is a severe but treatable condition that rarely complicates Hashimotos thyroiditis. Clinically it is characterized by progressive or relapsing symptoms, including tremor, myoclonus, stroke-like episodes, seizures, impairment of consciousness, and dementia. We describe a patient presenting with recurrent generalized convulsive status epilepticus (GCSE), despite antiepileptic medications, who was successfully treated with methylprednisolone. Our observation confirms that the clinical spectrum of HE at presentation is heterogeneous and diagnosis is often difficult. This case highlights the crucial importance of antithyroid antibody measurement in patients presenting with otherwise unexplained episodes of GCSE with or without adjunctive signs of encephalopathy or thyroiditis.

Indole-3-Pyruvic Acid as a Possible Hypnotic Agent in Insomniac Subjects:

In a single-blind study six male patients (mean age 39.5 years) with moderate insomnia were treated with placebo for three nights, 100 mg indole-3-pyruvic acid (IPA) for three nights, 200 mg IPA for three nights, 100 mg IPA for two nights and placebo for two nights. Polygraphic recordings were made and total sleep time, sleep efficiency, sleep latency, slow wave sleep latency, rapid eye movement (REM) sleep latency, number of arousals (> 1 min), percentage and duration of wakefulness after sleep onset, percentage and duration of wakefulness after sleep onset, percentage and duration of sleep stages 1, 2, 3, 4 and REM were recorded. At the end of 13 days, total sleep time, duration of stage 2 sleep and total non-REM were significantly increased when compared with baseline. Total sleep time and duration of stage 2 and total non-REM sleep on completion were significantly decreased when compared with after 200 mg IPA (night 9). Results suggest an action of IPA on human sleep similar to that of exogenous melatonin and l-tryptophan, thus confirming that IPA could be used to increase serotonin and melatonin turnover.

Generalized EEG abnormalities in cluster headache

Sir, A strict temporal association between cluster headache (CH) attacks and sleep has been described [1], but very few polysomnographic studies of CH patients during their active period have been undertaken. For this reason, we performed polysomnography in thirteen unmedicated patients (9 males, 4 females; mean age 41.38 years) affected by episodic CH (IHS criteria, 2) and with ocular autonomic signs ipsilateral to the side of pain. Cluster attacks occurred during the night recording in only three patients (2 males, 1 female); three females, none of whom experienced attacks during the night, presented generalized EEG abnormalities. Of these latter patients, patient 1, aged 29 years, has suffered from photosensitive GM seizures, well controlled by phenobarbital, since the age of 22; her CH started when she was 24. Baseline EEG showed generalized photoparoxysmal discharges whereas, during NREM sleep, EEG activity was characterized by brief trains of generalized spike and polyspike and wave complexes (3 c/sec). Patient 2, aged 37 years, has a positive familial history of idiopathic epilepsy and suffered a head injury at the age of 24. Clinical, EEG and neuroradiological findings had always been normal. CH started at the age of 28. The nocturnal recording revealed frequent trains of generalized rapid spike and polyspike and wave complexes (3-4 c/sec), strongly activated by all NREM sleep stages. Patient 3, aged 41 years, with no familial or personal history of neurological disorders, has experienced CH since the age of 20. Her NREM sleep EEG showed dispersed generalized rapid (4-5 c/sec) polyspike and wave complexes bilaterally predominant on the anterior leads. In patient 1, the EEG findings may be explained by her known generalized epileptic seizures; an association between CH and epilepsy has recently been reported [3]. The ipsilateral association of pain attacks with head injur,

Serotoninergic agents in the treatment of Gilles de la Tourette's syndrome.

(case 2) has also been reported [4]. However, the third patient had no antecedents to explain her LEG pattern. Our findings might be an element in favour of the central hypothesis of CH and the already described possibility of a gender relationship [5]; studies of a larger sample of patients, with a careful follow-up that includes the remission phase of CH, would be advisable to confirm observation.