resolutionmini

haut de page

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

haut de page

mise à jour du
15 août 2002
Mov Disord
1996;11(6):738-740
1997;12;(5):819
lexique
Paroxysmal kinesigenic dystonia associated with a medullary lesion
David E Riley
Movement Disorders Center, Mt. Sinai Medical Center and Department of Neurology, Case Western Reserve University School of Medicine, Cleveland, Ohio
 

Chat-logomini
Paroxysmal kinesigenic dystonia (PKD), or choreoathetosis (PKC), is a transient movement disorder characterized by its precipitation by initiation or acceleration of movement, a duration of usually <1 min and always <5 min, a high frequency of 100 or more episodes per day and a striking benefit from anticonvulsants (1-3). The most common precipitating movement is rising quickly from a seated position (1). Most paroxysms are preceded by sensory prodromata and followed by a refractory period (3). In the majority of cases, the movements affect the limbs in a unilateral fashion (1,3). Following is a description of a case conforming to the clinical criteria of PKD outlined above, but with a unique distribution limited to the territory of lower cranial nerves and associated with a lesion in the medulla.

CASE REPORT : A 69-year-old man complained of spasms of his throat, which had hegun abruptly 2 years earlier. These episodes consisted of tightening of the throat muscles which felt like golf balls on either side of his throat. This was accompanied by elevation of the tongue to the palate. The net results were dysphonia and dysarthria. During 25% of episodes he simultaneously felt a burning sensation around the eye, radiating down over the check and the side of the neck; he estimated that the right side was involved four times as often as the left, but never both sides at once. The episodes occurred six to 20 times per day and lasted 10-30 s each, resolving rapidly.

Spasms were provoked by a number of activities, most notably speech, especially at initiation. At times, anticipation of speech by picking up the receiver or just hearing the telephone ring could provoke a spasm. Other mouth actions could cause an episode to occur, including chewing food (particularly the first bite), swallowing, brushing teeth, and yawning. Occasionally, whole body motions such as arising from a chair or lifting a heavy weight would also result in a spasm. Immediate repetition of the provocative action after a spasm would never result in a second episode, and he could resurne speaking, chewing, or lifting without further incident. He had been treated with clonazepam up to 2.5 mg/day, producing only disequilibrium.

His medical history was notable for hypercholesterolemia, ischemic heart discase, and a coronary artery bypass graft 10 years previously. He had also undergone a prostatectomy for benign prostatic hypertrophy and a lumbar diskectomy. His daily medications were bethanccol, simvastatin, and aspirin. The family history disclosed no neurologie illness.

Results of the general neurologie examination were unremarkable. His speech at rest was normal. Two episodes of throat spasms were produced by sitting up from a supine position, and one by drinking (videotape). Objectively, these consisted of a strained dysphonia and palpable hardening of supralaryngeal muscles bilaterally, lasting -15 s. Speaking, eating, or answering the telephone produced no reaction.

A magnetic resonance imaging (MRI) scan of the brain demonstrated a medullary lesion consistent with a remote hemorrhage. No abnormal blood vessels were seen. On two subsequent office visits, he developed throat spasms upon arising from his chair in the waiting room. The only other episode was provoked by drinking water. He began treatment with phenytoin 300 mg every morning. He stopped having attacks the following day. When he reduced his daily dosage to 200 mg, he felt a rare sensation of tightness in his jaw "almost like it was trying to do it again." However, he has had no throat spasms since starting phenytoin 3 years ago.

DISCUSSION : This patient's story is typical of PKD in every respect save for the distribution of the muscles affected. PKD almost always involves the limbs, usually in a unilateral fashion; no prior case of PKD of the pharynx larynx and tongue has been reported. The anatomic location of the lesion shown suggests strongly that it is responsible for the unique clinical presentation because the cranial nerve nuclei (ambiguus, hypoglossal) predominantly responsible for motor innervation of the pharynx, larynx and tongue are located at the medullary level.

The mechanism for production of PKD is unknown. Most investigators speculating on this topic have chosen to regard PKD as either a disorder originating in the basal ganglia (1,4) or a form of reflex epilepsy (5,6). The evidence in favor of a basal ganglia lesion includes the dystonic (or choreic or athetotic) character of the involuntary movement, the coexistence in a minority of patients of other dyskinesias suggesting basal ganglia disease, and a lone report of a patient responding to levodopa (7). Kertesz reasoned that PKD, at least in idiopathic or familial cases, could represent "a paroxysmal release phenomenon from striatal control- caused by "i nsufficient maturation of some of these control pathways, leading to pathological oscillation in the circuits of the hasal gangli due to a disordered feedback mechanism (1).

Support for the notion of PKD as an epileptic phenomenon comes from its episodic, stereotyped occurrence, analogous to that of seizures, and its response to anticonvulsants. Features inconsistent with epilepsy are the preservation of consciousness, even during bilateral episodes, and the conspicuous absence of paroxysmal epileptiform activity from electroencephalograph recordings during attacks. Franssen et al found an enhanced cortical potential (the "slow negative wave") in an electrophysiologic study of the contingent negative variation after a signal warning of anticipated tasks in a patient with PKC (8). However, they were careful to conclude that the electrical abnormality could have arisen from a disturbance in an unknown part of the "mediothalamic-prefrontocortical-nucleus reticularis thalami system," and its relationship to PKC was not established.

Most cases of secondary or symptomatic PKD are due to multiple sclerosis or brain trauma (9) and cannot be correlated with a specific focal lesion of the central nervous system. The small number that can be assigned a localization show a diversity of clinical-pathological correlation. PKD is associated with focal lesions at all levels of the neuraxis, not just within the cerebral cortex or basal ganglia but also in the thalamus, brainstem, and spinal cord. This suggests that PKD is not a manifestation of dysfunction within a specific region or pathway of the nervous system, but rather a shared expression of disorders of varying etiology and location, whose distribution depends on the site of the lesion. Details of the pathophysiology remain obscure, but fundamentally there appears to be a disordered response to movement or anticipation of movement. The occurrence of dyskinesias with anticipation, and preceding actual movement, indicates that the aberrance occurs in the planning stage of actions rather than as a result of the influx of sensory information from the movement itself, such as proprioceptive changes. At the very least, patients such as the one described here compel us to broaden our consideration of the pathophysiology of PKD beyond the scope of epilepsy and basal ganglia disease.

REFERENCES

  1. Kertesz A. Paroxvsnial kinesigenic choreoathetosis. Neurology 1967;17:680-690.
  2. Lance JW. Familial paroxysmal dystonic choreoathetosis and its differentiation front related syndromes. Ann Neurol l977;2:285-293.
  3. Goodenough DJ. Fariello RG, Annis BL, Chun RW. Familial and acquired paroxysmal dyskinesias. Arch Neurol l978~ 35:827--831.
  4. Smith LA, Heersema PH. Periodic dystonia. Staff Meet Mayo Clin 1941;16:842-846.
  5. Lishman WA. Symonds CP, Whitty CW, Wilson RG. Seizures induced by movement: a form of reflex epilepsy. Brain 1962;85:93-108.
  6. Stevens H. Paroxysmal choreo-athetosis: a form of reflex epilepsy. Arch Neurol 1966;14:415-420.
  7. Loong SC, Ong YY. Paroxysirial kinesigenic choreoathetosis: report of a case relieved by L-dopa. J Neurol Neurosurg Psychitry 1973;36:921-924.
  8. Franssen IL Forigens C. Wattendorff AR, van Woerkom TC. Paroxysmal kinesigenic choreoathetosis and ahnormal contingent negative variation. Arch Neurol 1983;40:381-385.
  9. Fahn S. The paroxysmal dyskinesias. In: Marsden CD, Fahn S, eds. Movement disorders 3. Oxford, England: Butterworth-Heinemann: 1994:310-345.
  10. Falconer MA. Driver MV, Serafetinides EA. Seizures induced by movement: report of a case relieved by operation. J Neurol Neurosurg Psychitry, l963;26:30(~-307.
  11. Richardson JC, Howes JL, Celinski MJ, Allman RG. Kinesigenic chorcoathetosis due to brain injury. Can J Neurol Sci 1987; 14:626--628.
  12. Merchut MP. Brumlik J. Painful tonic spasms caused by putaminal infarction. Stroke 1986:17:1319-1321.
  13. Burguera JA, Catala J, Casanova B. Thalamic demyelination and paroxysmal dystonia in multiple sclerosis [Letter]. Mot, Disord 1991:6:379-381.
  14. Lee MS, Marsden CD. Movement disorders following lesions of the thalamus or subthalamic region. Mov Disord l994~9:493-507.
  15. Camac A. Greene P, Khandji A. Paroxysmal kinesigenic dystonic choreoathetosis associated with a thalamic infarct,Mov Disord 1990:5:235-238.
  16. Hutchinson M. Bresnihan B. Neurological lupus erythematosus with tome seizures simulating multiple sclerosis. J Neurol Neurosurg Psvchiaitry, 1983;46:583-585.
  17. Blankenship L. Newton HB. Cervical myelitis and paroxysmal dystonia causeed by herpes simplex virus type 1 Abstract Ann Neurol 1992:32:264.
Comprendre avec l'aide de K Ezure la coordination respiration - marche
Convergence of central respiratory and locomotor rhythms onto single neurons of the lateral reticular nucleus Ezure K, Tanaka I Exp Brain Res1997; 113; 2; 230-242