Proprioceptive-induced seizures

Proprioceptive-induced seizures
Benajmin G. Zifkin and Frederick Andermann
Date of submission: July 20, 2001
Date of MEDLINE search: July 2001

HISTORICAL NOTE AND NOMENCLATURE

Reflex attacks apparently induced by movement were reported almost 100 years ago (Gowers 1901). Although early reports described attacks apparently induced by movement (Lishman et al 1962), later work demonstrated the paramount role of proprioceptive afferents (Chauvel and Lamarche 1975). Thus, seizures originally described as movement-induced or gait-induced are usually more accurately described as "proprioceptive-induced”. These reflex seizures are rare, though well-described, and recently have been reviewed by Vignal and colleagues (Vignal et al 1998). Proprioceptive-induced seizures are included as a reflex seizure type in the most recent proposed classification of epilepsy syndromes. In this proposal, reflex epilepsy syndromes are those ". . . in which all epileptic seizures are precipitated by sensory stimuli. Reflex seizures that occur in focal and generalized epilepsy syndromes that are also associated with spontaneous seizures, are listed as seizure types” (Engel 2001).

CLINICAL MANIFESTATIONS

Proprioceptive-induced seizures are evoked by passive or active movement without startle. The seizures are usually brief tonic seizures or simple partial attacks induced by active or passive movement of a limb and usually occur in subjects with fixed cerebral lesions and motor deficit. They may begin with a jacksonian pattern of sensory manifestations. They have been described as a transient phenomenon during nonketotic hyperglycemia, resolving with metabolic correction (Brick et al 1989) and as self-induced seizures with compulsive proprioceptive self-stimulation (Guerrini et al 1992). The epileptic nature of these attacks has been confirmed by ictal EEG recording (Arseni et al 1967). Reflex drop attacks can occur with walking in patients with EEG vertex spikes evoked by percussion of the sole of the foot; most subjects with this unusual EEG finding do not have such seizures (Tassinari et al 1988).r types of generalized seizures (Panayiotopoulos et al 1995).

LOCALIZATION

Proprioceptive-induced seizures classically involve the rolandic sensorimotor area of the hemisphere contralateral to the clinical seizure onset. The supplementary motor area may also be involved. This localization has been confirmed by imaging and intensive monitoring. Cerebral lesions are often evident and may have occurred well before the onset of attacks. In cases with nonketotic hyperglycemia, an associated neurologic deficit related to a remote lesion may be transiently unmasked during the period of seizures (Brick et al 1989). Acute cerebral lesions may also be accompanied by self-limited proprioceptive-induced seizures.

PATHOPHYSIOLOGY

Proprioceptive-induced seizures have been studied with a chronic alumina focus in one foot area of the monkey brain. Reflex seizures were elicited by stimuli that produced proprioceptive input to the hyperexcitable cortical area. Seizures could not be elicited after curarization (Chauvel and Lamarche 1975). Transcortical reflex loops appear to be involved in generating the proprioceptive-induced motor response in the cat with a penicillin focus (Giovanni et al 1983). Human proprioceptive-induced seizures are seen during nonketotic hyperglycemia, in which attacks are induced by active or passive limb movement and more rarely with conjugate gaze (Brick et al 1989; Duncan et al 1991). Many patients have fixed or transient neurologic deficits, suggesting a mechanism similar to that described for the monkey.

DIFFERENTIAL DIAGNOSIS

Proprioceptive-induced seizures should not be confused with startle epilepsy. In subjects with gross cerebral damage, the nature and localization of brain lesions are similar in the 2 syndromes, but the nature of the triggering stimulus is quite different. Proprioceptive-induced seizures begin less suddenly and may have initial jacksonian sensory manifestations. They can usually be distinguished from paroxysmal kinesigenic dystonia (choreoathetosis), characterized by dystonic and choreoathetoid movements, preserved consciousness, and a normal EEG during brief attacks that are rapidly induced by movement (Cler et al 1990). Paroxysmal kinesigenic dystonia is often familial, begins in neurologically normal children or young adults without evident lesions, and may be a channelopathy (Bhatia 1999). Spontaneous improvement often occurs.

DIAGNOSTIC WORKUP

Proprioceptive-induced seizures have been reported as a distinct syndrome with nonketotic hyperglycemia. Acutely ill patients with these seizures require urgent investigation for this and other common encephalopathies. Recent or remote cerebral lesions often coexist with acute-onset proprioceptive-induced seizures. CT or MRI studies are urgently indicated in all such patients. Other patients with chronic proprioceptive-induced seizures require imaging studies to localize any treatable lesion that may be responsible for the attacks. Such lesions may be small, and detailed MRI may be needed. The history will allow tailoring of the EEG investigation. Seizures can be induced easily for study in the EEG laboratory, although reduction of medication may be needed. If proprioceptive activation is suspected in drop attacks, EEG monitoring of percussion of the sole of the foot with a reflex hammer (Tassinari et al 1988) may be helpful. This easy and brief test is worth performing in patients suspected of proprioceptive-induced seizures.

SYNDROMES AND DISEASES IN WHICH THE SEIZURE TYPE OCCURS

Chronic proprioceptive-induced seizures in medically stable patients are usually manifestations of remote nonprogressive lesions as described above. As in other patients with seizures, a progressive lesion such as tumor must be excluded. Chronic (Rasmussen) encephalitis may also give rise to proprioceptive-induced seizures.

Proprioceptive-induced seizures have been reported with nonketotic hyperglycemia.

PROGNOSIS AND COMPLICATIONS

Proprioceptive-induced seizures without acute illness are rare and their prognosis unclear. Those seen with nonketotic hyperglycemia resolve with successful treatment of the hyperglycemia. New onset proprioceptive-induced seizures with acute cerebral lesions may cease over days or weeks on their own and may require no specific treatment.

MANAGEMENT

Proprioceptive-induced seizures are rare, and there is no consensus as to their treatment. Carbamazepine would be a rational first choice, possibly with the addition of clobazam (not available in the United States) if needed. Surgery has been reported as effective, but there has been no published series evaluating outcome in such patients (Falconer et al 1963).

REFERENCES CITED

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