Sleep Studies and Therapeutic Trial with L-Dopa in a Case of Stiffman Syndrome

Abstract

A case of Stiffman syndrome was followed for several years. A trial with L-dopa was performed after diazepam withdrawal. All-night polygraphic monitorings of the patient’s sleep under diazepam, under no drugs, and under L-dopa were performed. An analysis of sleep stages and electromyographic changes was obtained. A new benzodiazepine (Ro 054023) was tried after L-dopa. A possible imbalance between a catecholaminergic and a GABA-ergic system in the Stiffman syndrome is hypothesized.

Stiff-man syndrome associated with nocturnal myoclonus and epilepsy.

“…The myoclonus in our case seems similar to the rhythmic spasms described by Guilleminault et al (1973). Lugaresi et al (1968) reported on a number of cases of nocturnal myoclonus associated with spinal and supraspinal lesions.…”

“…Nor were there any modifications when glycine was administered, thus excluding the hypothesis of a dysfunction of the inhibitory glycinergic spinal interneurones (Schmidt et al, 1975). L-dopa worsened the rigidity (Guilleminault et al, 1973;Schmidt et al, 1975) while diazepam, clonazepam, and baclofen reduced the spasms (Howard, 1963;Mertens and Ricker, 1968;Guilleminault et al, 1973). On the basis of these pharmacological data, the hypothesis has been formulated that the stiff-man syndrome depends on an imbalance between the cholinergic systems (whose activity is increased by L-dopa and depressed by benzodiazepines) and the GABA system (whose activity is increased by baclofen) (Guilleminault et al, 1973;Schmidt et al, 1975).…”

“…Berti Ceroni et al (1967) observed a particular abundance of non-repetitive myoclonic jerks during REM sleep, localised to the limb predominantly affected by contractions during wakefulness, which seemed to be an intensification of the physiological myoclonic twitches. Guilleminault et al (1973) observed "paroxysmal spasms," recurring every 10-45 seconds during phase 1 sleep, every five to eight minutes during phase 2, and disappearing completely during phase 3 and REM sleep. In our patient nocturnal polygraphic recording showed the existence of a typical nocturnal myoclonus (Symonds, 1953) consisting of muscular jerks of the legs occurring every 15-20 seconds during light sleep.…”

Stiff Person Syndrome

“…In the earliest descriptions of jerking SPS, patients had a protracted history of progressive appendicular and axial rigidity with spasms, identical to classical SPS, before developing nocturnal myoclonus [114,115]. The term 'jerking stiff man syndrome' did not emerge until the 1980s [116,117] and the condition is now generally referred to as jerking SPS.…”

A new syndrome of axial muscle rigidity associated with etretinate therapy

“…In addition, the stiff-man syndrome has been reported to worsen with L-Dopa administration (10) and improve with clonidine (1 I), an alpha-2 agonist which reduces catecholamine release.…”