| Epilepsia partialis continua by Heinz Gregor Wieser Date of submission: February 20, 2001 Date of update: January 2002 Date of update: February 28, 2003 Medline SEARCH DATE: February 2001 |
| HISTORICAL NOTE AND NOMENCLATURE The presentation of the disorder depends on the underlying cause. Patients with localized neoplastic, vascular, or infectious brain lesions may have neurologic deficits and isolated seizures prior to the onset of the focal status (Bancaud 1985). With metabolic causes, however, such as nonketotic hyperglycemic diabetes mellitus, or hypersensitive reactions to certain drugs such as penicillin or metrizamide, the onset of focal status epilepticus is sudden (Schomer 1993). In the Russian patients, the epilepsy usually developed
3 to 4 months after a febrile illness, associated with a hemiplegia or
monoplegia in 30% of cases. In this condition the jerks typically affect
agonist and antagonist muscles with a rhythmic quality, in short bursts
of 1 to 2 seconds’ duration alternating with quiescent phases 2
to 4 seconds long, persisting in sleep and worsened by action or stress.
Motor seizures with Jacksonian march or generalized epileptic seizures
are almost invariable accompaniments, although with a strong tendency
to improve over time. The jerking, often highly focal, continues relentlessly
for years. Sensory symptoms occur in about one-fifth of cases, and 80%
have a persisting hemiparesis. Epilepsia partialis continua associated
with Rasmussen encephalitis manifests itself in children in the majority
of cases (mean age 6.8 years with 85% being under the age of 10 years).
All children present with epileptic seizures, often generalized tonic-clonic,
although focal simple or complex partial seizures also occur as a first
manifestation of the disease. In Rasmussen encephalitis, about half of
the patients exhibit episodes of epilepsia partialis continua, usually
within 3 years of onset with epilepsy. These episodes last hours to years
and are often discontinuous. The condition is progressive, and after a
highly variable period of 3 months to 10 years, fixed focal deficits develop,
notably hemiplegia, hemianopia, and (depending on the hemisphere) aphasia,
as well as progressive intellectual impairment. After an initial progressive
course, in which the focal motor seizure activity is often multifocal
motor, the disease process appears to eventually burn itself out, at least
in a substantial proportion of Rasmussen encephalitis patients. Current views are that the physiological characteristics of the jerks in most cases of epilepsia partialis continua are identical to those of cortical myoclonus (see pathophysiology). In the past, though, an influential paper by Juul-Jensen and Denny-Brown reported the electrophysiological and pathological details of 9 patients with acute (mostly large) cerebral lesions with subcortical damage (albeit in most cases coexisting cortical motor area damage) (Juul-Jensen and Denny-Brown 1966). This paper created a controversy regarding cortical versus subcortical origin and the differentiation from myoclonus (Shorvon 1994). The myoclonic jerks in epilepsia partialis continua can affect any muscle group. They may be confined to a single muscle or muscle group or they may be widespread. The distribution of jerks can vary over time. Agonists and antagonists are affected together, and distal muscles are affected more often than proximal. Face, upper limb, and trunk predominate. Jerks are unilateral. Bilateral cases have been included (Lohler and Peters 1974; Thomas et al 1977), but it is questionable whether such cases should be described within the category called epilepsia partialis continua. Takahashi and colleagues (Takahashi et al 1997) have studied epilepsia partialis continua of childhood involving bilateral brain hemispheres, and Ashkenazi and colleagues have described a bilateral focal motor status epilepticus with retained consciousness after stroke (Ashkenazi et al 2000). Familial alternating epilepsia partialis continua with
chronic encephalitis as another variant of Rasmussen syndrome has been
described by Silver and colleagues (Silver et al 1998); Yacubian and colleagues
(Yacubian et al 2001) described Rasmussen encephalitis associated with
segmental vitiligo of the scalp. However, absence of epileptogenic EEG abnormalities in some patients with epilepsia partialis continua (Penfield and Jasper 1954) and presence of subcortical brain lesions with preserved cortex (Juul-Jensen and Denny-Brown 1966; Botez and Brossard 1974) led to the hypothesis of a subcortical origin of the epilepsia partialis continua in at least some patients. In 1985 Hallett introduced 3 types of epileptic myoclonus: (1) cortical reflex myoclonus as a fragment of partial epilepsy, which represents hyperactivity of a focal area of cerebral cortex; (2) reticular reflex myoclonus as a fragment of generalized epilepsy with hyperactivity of medullary brainstem reticular formation; and (3) primary generalized epileptic myoclonus as a fragment of primary generalized epilepsy, which may represent a generalized hyperactive response of cortex to subcortical input (Hallett 1985). In cases with cortical reflex myoclonus, the epileptogenic focus is localized in the contralateral rolandic cortex, and the EEG may show spikes related to the myoclonic jerks. In cases without a clear-cut temporal relation between epileptic events and myoclonus in the EEG, the back-averaging technique identifies the spikes preceding the myoclonus (Shibasaki and Kuroiwa 1975). Somatosensory evoked potentials of the rolandic cortex are abnormally enlarged (Shibasaki et al 1978). Without such proof of the cortical origin of the myoclonus by neurophysiological methods, a subcortical, or even spinal origin of the myoclonus has to be considered. Recently, Cockerell and colleagues suggested that the diagnosis of epilepsia partialis continua should be confined exclusively to cortical myoclonus (Cockerell et al 1996). The authors proposed the term "myoclonia continua" for myoclonus that arises extracortically. In cases of epilepsia partialis continua where it is
associated with other seizures, the physiological characteristics of the
jerks are identical to those of cortical myoclonus (Shorvon 1994). Cortical
myoclonus can be viewed as a hypersynchronous discharge from a group of
cortical cells. In this sense, it is cortical epilepsy. Concerning myoclonus
in general, by employing different physiological methods, such as back-averaging
of EEG recordings in relation to the jerks, evoked potentials, and electromyographic
recordings of the sequence of recruitment of muscle groups in a myoclonic
jerk, a distinction of myoclonus into cortical, brainstem, and spinal
myoclonus is possible in most cases. Typically in cortical myoclonus,
back-averaged time-locked EEG cortical generator potentials precede the
jerks; sensory evoked potentials are enlarged; and the myoclonus may be
spontaneous and action- or stimulus-sensitive with a rostrocaudal pattern
of muscle recruitment and antagonist and agonist co-contraction. In stimulus-sensitive
myoclonus, the reflex timings are compatible with a cortical loop. Obeso
and colleagues report patients showing a spectrum of spontaneous and stimulus-sensitive
myoclonus, epilepsia partialis continua, Jacksonian seizures, and generalized
seizures, all with similar physiology (Obeso et al 1985). In these cases
it is hard to escape the view that epilepsia partialis continua is simply
repetitive cortical myoclonus. However, in cases without seizures other
than epilepsia partialis continua, jerks resemble epilepsia partialis
continua clinically but not neurophysiologically. In these cases the jerks
might be of subcortical origin. Menini and Naquet called this variant
type C myoclonus with suggested origin in the brainstem (Menini and Naquet
1986). It is, however, fair to say that this variety is not as common
nor as well studied as the cortical myoclonus, and its exact nosological
position is not clear. The differential diagnosis of myoclonus is difficult. The Commission on Pediatric Epilepsy of the ILAE grouped myoclonus into (1) cortical myoclonus, (2) thalamocortical myoclonus, (3) reticular reflex myoclonus, and (4) negative myoclonus (Commission on Pediatric Epilepsy of the ILAE 1997). According to this Commission report, epileptic myoclonus should be distinguished from:
Myoclonus may combine with epilepsy in various conditions:
Epileptic negative myoclonus may be generalized or focal. Epileptic negative myoclonus is a heterogeneous condition that may originate from various brain areas, including premotor cortex and motor cortex. It may be correlated with the slow wave of a spike-wave complex (as in the syndrome of continuous spike waves during slow-wave sleep) or with the negative transient of the polyspike of a polyspike-wave complex (as in myoclonic absence). Symptomatic generalized epileptic negative myoclonus due to Lance-Adams syndrome may be combined with positive myoclonus. Asterixis was reported to be misdiagnosed for an epilepsia partialis
continua (Stell et al 1994), as was Parkinson disease (Al-Hayk and LeDoux
2003). Dystonia, athetosis, and epilepsia partialis continua was reported
in a patient with late-onset Rasmussen encephalitis (Frucht 2002). Andermann
and colleagues described the syndrome of prolonged classical migraine,
epilepsia partialis continua, and repeated strokes as a clinically characteristic
disorder probably due to mitochondrial encephalopathy (Andermann et al
1986). Varlamov and colleagues (Varlamov et al 2002) have identified a
novel heteroplasmic C6489A missense mutation in the mitochondrial DNA
(mtDNA) CO I gene encoding the cytochrome c oxidase subunit I in a 17-year-old
girl with epilepsia partialis continua. Relatively characteristic, other pathological findings in MRI and MR-spectroscopy as well as pathological SPECT and PET findings may be helpful for the early diagnosis and particularly for targeting the often intended brain biopsy. A moderately to severely abnormal EEG with progressive slowing and spiking is the rule. It is important to note that CSF may be abnormal with elevation of protein and lymphocytes, but a normal CSF does not rule out the presence of Rasmussen encephalitis. Kim and colleagues (Kim et al 2002) studied 7 children with Rasmussen syndrome in a prospective longitudinal MRI study with 3 to 8 MRIs per patient performed between 12 months before and 9 months after the onset of epilepsia partialis continua. These authors described that the most common region of initial MRI signal change was the frontocentral region (6 patients). Three patterns of neuroimaging abnormalities were observed as follows:
Park and colleagues (Park et al 2000) performed magnetic resonance spectroscopy
in 3 pediatric patients with epilepsia partialis continua measuring the
spectral peaks of several metabolites (N-acetyl-aspartate, choline, creatine,
and lactate) and observed increased lactate-to-creatine ratios and reduced
N-acetyl-aspartate-to-creatine ratios in the affected hemispheres in all
3 children with epilepsia partialis continua. These data support previous
reports. Kozhevnikov suggested, without neuropathological evidence, that the myoclonic jerks originate in the cerebral cortex due to a localized encephalitis (Kozhevnikov 1895). Thirty years later, Omorokow proved the Kozhevnikov hypothesis in a series of 52 patients by performing numerous cortical biopsies (Omorokow 1927). Today, Kozhevnikov cases are believed to have been due to an infectious agent known as Russian spring summer encephalitis (Zemskaya et al 1991). Bancaud and colleagues delineated 2 epilepsia partialis continua syndromes (Bancaud et al 1982). These authors assigned the epilepsia partialis continua type I to a localized pathology of the rolandic cortex, whereas epilepsia partialis continua type II was assigned to a diffuse unilateral encephalitic process. Encephalitic epilepsia partialis continua is the most frequent form in childhood and is related to Rasmussen syndrome (Rasmussen et al 1958). In short, the clinical picture of this "chronic encephalitis" is characterized by a severe focal seizure tendency beginning in infancy and childhood, often associated with epilepsia partialis continua. Patients with epilepsia partialis continua show a slowly progressive neurologic deterioration, usually hemiparesis and mental retardation, which advances over periods of months or years before the progression becomes arrested (Oguni et al 1991). With few exceptions, the pathological process with a gradual destruction of brain tissue involves one hemisphere only. The majority of patients with Rasmussen encephalitis exhibit an inflammatory episode of some sort at, or shortly before, the onset of seizures. In relatively well-preserved brain areas, perivascular lymphocytic cuffs and glial nodules, and in later stages microcystic degeneration with marked neuronal fallout but without evidence of inflammatory elements are typical histological findings (Robitaille 1991). It is fair to say that the nature of this disease remains obscure, although in recent years an autoimmune process has been postulated, as prompted by various reports (Rogers et al 1994; Twyman et al 1995; Andrews et al 1996). Autoantibodies in sera from patients with active Rasmussen encephalitis and experimentally-induced antibodies in rabbits seemed to act as agonists for glutamate receptors consisting of or containing GluR3 subunits. Agonist activity of autoantibodies on a glutamate receptor subunit suggested their role as pathogenetic factors, potentially as highly specific excitotoxins or neuromodulators. Our search, however, for the presence of anti-GluR3 antibodies in sera and CSF of 4 patients with Rasmussen encephalitis yielded negative results (Tonnes et al 1998). Today it is generally accepted that epilepsia partialis continua may be associated with focal, multifocal, and diffuse brain lesions and may include numerous syndromes. In children, besides the Rasmussen encephalitis, the other main causes for an epilepsia partialis continua are multisystem degenerative diseases, such as mitochondrial disorders (Andermann et al 1986; Carrascosa et al 1990; Antozzi et al 1995; Veggiotti et al 1995), or the Alpers syndrome (Wilson et al 1993; Worle et al 1998; Rasmussen et al 2000). In adults, epilepsia partialis continua is most frequently related to atherosclerotic cerebral vascular diseases, strokes, and tumors. Less frequent causes are metabolic disturbances, such as nonketotic hyperglycemic diabetes mellitus, particularly associated with hyponatremia (Singh and Strobos 1980), renal and hepatic encephalopathy (Morres and Dire 1989), and cortical dysplasia (Nordborg et al 1987; Desbiens et al 1993). Epilepsia partialis continua has also been associated with multiple sclerosis, mitochondrial encephalopathy with lactic acidosis and strokes, mitochondrial encephalopathy with ragged red fibers, and MELAS plus syndrome (Peterus et al 1997). Epilepsia partialis continua as the first clinical manifestation has been described in progressive cerebral degeneration of childhood with liver disease (Alpers Huttenlocher disease) with cytochrome oxidase deficiency (Worle et al 1998) as well as in a patient with a missense mutation in the mitochondrial DNA CO I gene encoding the cytochrome c oxidase subunit I (Varlamov et al 2002). This point mutation leads to an exchange of the highly conserved Leu196 to Ileu196. Muscle biopsy showed in single fibers decreased cytochrome c oxidase activity and lowered binding of cytochrome c oxidase antibodies, indicating decreased stability of the mutated enzyme. The analysis of blood mtDNA revealed about 30% mutant mtDNA in the patient's blood. Epilepsia partialis continua has been observed in Creutzfeldt-Jakob disease (Lee et al 2000; Parry et al 2001) and succeeding bone marrow transplants (Antunes et al 2000). It was observed in association with widespread gliomatosis cerebri (Shahar et al 2002), as an atypical presentation of cat scratch disease in a young adult (Nowakowski and Katz 2002), in association with type 1 diabetes mellitus and elevated anti-GAD65 antibodies (Olson et al 2002), in ketotic and nonketotic hyperglycemia (Placidi et el 2001; Sabitha et al 2001), and in HIV-infected patients (Ferrari et al 1998; Bartolomei et al 1999). Kufs disease presented as late-onset epilepsia partialis continua (Gambardella et al 1998). Epilepsia partialis continua has been described in association with a homoplasmic mitochondrial tRNA (Ser(UCN)) mutation (Schuelke et al 1998), as a new manifestation of anti-Hu-associated paraneoplastic encephalomyelitis (Shavit et al 1999; Porta-Etessam et al 2001). Epilepsia partialis continua has also been found in benign epilepsy
of childhood with centrotemporal spikes. Metrizamide, penicillin, and
azlocillin-cefotaxime may also induce this disorder (Schomer 1993). Antozzi and colleagues (Antozzi et al 1998) reported that long-term selective IgG immunoabsorption improves Rasmussen encephalitis; Dabbagh and colleagues (Dabbagh et al 1997) could stop seizures by intraventricular interferon-alpha in one case of Rasmussen encephalitis. In the case of Olson and colleagues (Olson et al 2002), with the diagnosis of type 1 diabetes and antiglutamic acid decarboxylase 65 antibodies in his serum and cerebrospinal fluid, antiepileptic agents did not improve his seizures, but high-dose steroids, plasmapheresis, and intravenous immunoglobulin resulted in decreased antiglutamic acid decarboxylase 65 antibody levels and resolution of his seizures. At present, when the diagnosis of Rasmussen encephalitis is being considered,
it is important to rapidly exclude other causes of epilepsia partialis
continua. Although there are no good data from randomized trials of different
immune-related therapies, treatment with immunoglobulin G, steroids, or
plasmapheresis is advocated as first-line therapy. It is not unreasonable
to institute at least 2 treatment options (eg, IgG followed by plasmapheresis)
if response to the first treatment is poor (Counce et al 2001). Functional
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infectious or inflammatory etiology: recent surgical experience in the
USSR. In: Andermann F, editor. Chronic encephalitis and epilepsy. Boston:
Butterworth-Heinemann, 1991:271-82. ILAE ILAE Copyright Notice ABBREVIATIONS EEG:electroencephalography SYNONYMS MAJOR KEYWORD DESCRIPTORS MINOR KEYWORD DESCRIPTORS AGE OF PRESENTATION AGE OF TYPICAL PRESENTATION GLOSSARY ILLUSTRATION CAPTIONS Figure 1 (not included because clinical vignette cut from seizure-oriented
structure--dlc) PERMUTED TOPIC, SYNONYMS, VARIANTS RELATED TOPICS Arboviral encephalitis DIFFERENTIAL DIAGNOSIS
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