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ORIGINAL ARTICLE
Year : 2013  |  Volume : 61  |  Issue : 2  |  Page : 156-160

Etiological profile of epilepsia partialis continua among adults in a tertiary care hospital


Department of Neurology, GMCH, Guwahati, Assam, India

Date of Submission04-Jan-2013
Date of Decision21-Jan-2013
Date of Acceptance22-Mar-2013
Date of Web Publication29-Apr-2013

Correspondence Address:
Ashok Kumar Kayal
Department of Neurology, GMCH, Indrapu Bhangagarh, Kamrup Metro, Guwahati - 781 032, Assam
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0028-3886.111122

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 » Abstract 

Background: Epilepsia partialis continua (EPC), is a subtype of status epilepticus, have a varied spectrum of etiology and the out-come depends on the etiology. Aims and Objectives: The present study is aimed to analyze the clinical characteristics and outcome. Materials and Methods: This is a prospective analysis of 17 patients admitted to our center between August 2010 and April 2012. EPC was defined as regular or irregular clonic muscular twitches affecting a limited part of the body, occurring for a minimum of 1 h, and recurring at intervals of no more than 10 s. The data collected included etiology, radiological findings, electroencephalogram (EEG) abnormalities, associated comorbid conditions, and outcome. Results: The mean age at presentation was 44.26 ± 13.77 years and the mean duration was 2.7 ± 1.5 days. There were ten patients with diabetic non-ketotic hyperosmolar state and one patient each of oligodendroglioma, varicella zoster vasculitis, central nervous demyelination, ischemic stroke, post traumatic seizure, arteriovenous malformation, and in one patient no cause could be established. Imaging showed abnormality only in five patients and EEG was abnormal in four patients. The EPC was controlled by one antiepileptic drug (AED) in eight patients, with two AEDs in seven patients and two patients required three AEDs. Conclusion: EPC is a rare type of focal motor status epilepticus. Treatment of the underlying cause in addition to controlling EPC is essential to achieve the good outcomes.


Keywords: Antiepileptic drugs, diabetic-non-ketotic-hyperosmolar-state, epilepsia partialis continua


How to cite this article:
Shrivastava V, Burji NP, Basumatary LJ, Das M, Goswami M, Kayal AK. Etiological profile of epilepsia partialis continua among adults in a tertiary care hospital. Neurol India 2013;61:156-60

How to cite this URL:
Shrivastava V, Burji NP, Basumatary LJ, Das M, Goswami M, Kayal AK. Etiological profile of epilepsia partialis continua among adults in a tertiary care hospital. Neurol India [serial online] 2013 [cited 2020 Feb 22];61:156-60. Available from: http://www.neurologyindia.com/text.asp?2013/61/2/156/111122



 » Introduction Top


Epilepsia partialis continua (EPC) is characterized by a simple partial motor seizure, restricted to one part of the body with repetitive regular or irregular clonic jerks without loss of consciousness. Clonic activity persists for days or weeks either continuously or intermittently and remains localized to a single muscle group or may even progress to secondary generalized convulsive status. EPC is uncommon with prevalence of less than one per million [1] EPC was first described by Kozhevnikov in 1985 (Kozhevnikov syndrome). EPC is presently considered as a type of seizure rather than a form of epilepsy and is categorized under focal status epilepticus. Bancaud et al.,[2] classified EPC into two types, type 2 being progressive and intractable. In the vast majority of cases, the seizures are of cortical origin; [3],[4] however, subcortical mechanisms also have been proposed. [5] The present study was aimed to analyze the etiology, radiological findings, electroencephalogram (EEG) correlates, comorbid conditions, therapeutic response and outcome in patients with the EPC as the presenting symptom.


 » Materials and Methods Top


This is a prospective study of 17 consecutive patients admitted to different wards of Gauhati Medical College Hospital, Gauhati, Assam, between August 2010 and April 2012. The criteria adopted for the diagnosis of EPC was those proposed by Thomas et al.: [4] "Regular or irregular clonic muscular twitches affecting a limited part of the body, occurring for a minimum of 1 h, and recurring at intervals of no more than 10 s" A detailed neurologic evaluation was carried out in all patients. Laboratory tests included complete blood picture, blood urea, serum creatinine, random blood sugar, serum electrolytes (sodium, potassium, calcium and magnesium), liver function tests, thyroid function tests, Human immunodeficiency virus (HIV) 1/2, urine routine/microscopy. All patients underwent neuroimaging, cranial computed tomography (CT) scan or magnetic resonance imaging (MRI) brain, and EEG. Selected patients underwent cerebrospinal fluid examination; serum osmolality was calculated using a formula: 2 (serum sodium) + (Glucose)/18+ (blood urea nitrogen [BUN])/2.8 where (Glucose) and (BUN) are measured in mg/dL and the serum osmolality more than 295 mOsm/L was considered as high. Urine for ketones was examined in patients with high blood sugar level.


 » Results Top


During the study period, the 17 (13 males and 4 females) patients were studied. The mean age was 44.26 ± 13.77 years (range 21-72 years). The mean duration of EPC was 2.7 ± 1.54 days (ranging 12 h-12 days). In 12 patients, EPC was localized to the right side, and in 5 to left side. EPC involved shoulder and hand more frequently than leg and distal more than proximal body parts and all patients were fully conscious during the entire period of EPC. In all the patients, sleep did not suppressed the EPC. All patients were alert at the time of hospitalization.

The etiological spectrum included [Table 1]: Diabetic non-ketotic hyperosmolar state (DNKHS) in 10 (58.8%) and 1 case each oligodendroglioma, varicella zoster vasculitis, central nervous system demyelination, ischemic stroke, traumatic brain injury, arteriovenous malformation and in one patient no cause could be established. Of patients with DNKHS group, in 4 (40%) patient's diabetes mellitus was detected de novo. The mean serum osmolality was 303.96 ± 13.17 mOsm/L (range 288.0-329.34 mOsm/L) mean random blood sugar at the time of the presentation was 608.30 ± 43.08 mg/dL (range 549.0-700 mg/dL). Hyponatremia was present in three patients at presentation (range 122-130.4 meq/dL) and five patients were alcohol dependent and two had a history of heavy alcohol intake before the onset of symptoms.
Table 1: Demographic profile of all patients with EPC

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Case 10 was a known case of diabetes with very poor glycemic control presented with continuous jerky movements of all four limbs with preserved consciousness of 1½ day duration and these movements were non-suppressible during sleep. Serum sodium was 122 meq/dL [Table 1]. He also had a history of alcohol intake about 6 h prior to the onset these movements. The seizures were controlled with proper glycemic control and intravenous fosphenytoin and clobazam.

The patient with varicella zoster vasculitis (case 5) presented with EPC involving right hand and leg, which improved gradually. He was sero-positive for HIV infection.

Neuroimaging [Table 2]: CT scan was done in all patients and MRI brain in four patients. CT scan was abnormal in four patients: Oligodendroglioma (1), infarct (1) arteriovenous malformation (1) [Figure 1] and post traumatic focal gliosis (1). MRI brain was abnormal in two patients: Demyelination (1) and varicella zoster vasculitis (1) [Figure 2] and [Figure 3]. Neuroimaging was normal in all patients with DNKHS. EEG was obtained in interictal period in all patients, only 4(23.53%) patients showed epileptiform discharges. None of the patients of DNKHS group had an abnormal EEG [Table 2].
Figure 1: Computed tomography scan brain (plain) of case 17: Area of heterogenous density in Left parietal region suggestive of calcified arteriovenous malformation

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Figure 2: Magnetic resonance imaging brain of case 2: T2W hyperintense lesions in left frontal and parasagittal regions suggesting possibility of demyelinating plaques

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Figure 3: Magnetic resonance imaging brain of case 5: T2W hyperintense lesions in Left frontal white matter, left thalamus suggesting possibility of ischemic lesion (secondary to vasculitis)/demyelination

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Table 2: Investigation profile of patients with EPC

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All patients received single antiepileptic drug (AED) in the form of intravenous loading dose of fosphenyton, 20 mg/kg as the initial treatment and further AEDs were added when EPC could not be controlled with monotherapy. In 8 patients EPC was controlled by the initial AED, 7 patients required addition of second AED (clobazam), although 2 patients, one with oligodendroglioma and the other with arteriovenous malformation required 3 AED, valproate and levitiracetam). Two patients (post stroke and post traumatic) had second episode of EPC despite of good drug compliance, in both of them EPC was controlled by the addition of second AED.


 » Discussion Top


The first description of EPC, "epilepsia corticalis sive partialis continua," by Kozevnikov was in four patients with mild to moderate hemiparesis, in the paretic parts of the body. [6] He proposed that the origin of EPC was close to the primary motor cortex contralateral to the hemiparetic side; however he was unable to clarify the nature of the brain lesion, In view of the progressive nature of the disease in these patients, he hypothesized that probably a chronic encephalitic process might be at work. Probably he was right, these patients might had the tick-borne viral Russian spring summer meningoencephalitis, which was reported some decades later. [7] However, EPC has been reported in patients with a wide spectrum of etiologies. The International League against Epilepsy commission considers EPC as Kozhevnikov syndrome type 1 and defines it as "a particular form of rolandic partial epilepsy in both adults and children and is related to a variable lesion in motor cortex." The new diagnostic scheme recognized EPC as a seizure type and not as a syndrome. [8]

There are three large reported series of EPC. [3],[4],[9] The largest series, 76 cases, until the date is the study by Sinha and Satishchandra. [9] The etiological spectrum in this series included: Undetermined etiology (22.4%), stroke (19.7%), encephalitis (Rasmussen encephalitis: 9.2% and other infectious encephalitis: 10.5%), DNKHS (7.9%), and granuloma (7.9%). The etiological spectrum was also somewhat similar in the other two large studies. [3],[4] The etiological spectrum in a review of 162 patients with EPC [10] was inflammatory disorders in 32%, neoplastic disorders in 19%, head trauma in 16%, vascular disorders in 14%, others in 3%, and undetermined in 16%. In our series, the most common cause was DNKHS (58.8%). The discrepancy in the etiology could be related to case cohort, our study being in a medical college set up where the disease spectrum can be different than a specialty hospital set up. Serum hyperosmolality has been stressed as the cause of EPC in hyperglycemia. [11],[12] Experimental and clinical observations suggest that serum hyperosmolality and brain dehydration together increase the risk of seizures. [13] Similar were the observations in our study, mean serum osmolality was 303.96 ± 13.17 mOsm/L, and mean blood sugar was 608.30 ± 43.08 mg/dL. However, these observations in one of the large series were not similar. [11] When compared to the mean duration of EPC in the three large series, [3],[4],[9] the mean duration in our series was shorter, 20 days versus 2.70 days. The short duration of EPC observed in our series was mainly due to DNKHS in 58.8% of patients, which could be easily corrected within a short period. Alcoholism was comorbid factor in six patients, five of them in the DNKHS group. Heavy bout of alcohol intake prior to EPC was noted in two of the ten patients of DNKHS group.

EPC is a rare type of focal motor status epilepticus. Characteristic semiological features enable it to be diagnosed and distinguished from other movement disorders. The prognosis depends on the underlying cause and early treatment. The search for the underlying cause is of paramount importance. EPC can be the initial manifestation of diabetes mellitus.

 
 » References Top

1.Berkovic SF, Johns JA, Bladin PF. Focal seizures and systemic metabolic disorders. Aust N Z J Med 1982;12:620-3.  Back to cited text no. 1
    
2.Bancaud J, Bonis A, Trottier S, Talairach J, Dulac O. Continuous partial epilepsy: Syndrome and disease. Rev Neurol (Paris) 1982;138:803-14.  Back to cited text no. 2
    
3.Cockerell OC, Rothwell J, Thompson PD, Marsden CD, Shorvon SD. Clinical and physiological features of epilepsia partialis continua. Cases ascertained in the UK. Brain 1996;119:393-407.  Back to cited text no. 3
    
4.Thomas JE, Reagan TJ, Klass DW. Epilepsia partialis continua. A review of 32 cases. Arch Neurol 1977;34:266-75.  Back to cited text no. 4
    
5.Juul-Jensen P, Denny-Brown D. Epilepsia partialis continua. Arch Neurol 1966;15:563-78.  Back to cited text no. 5
    
6.Kozhevnikov AY. A peculiar type of cortical epilepsy (epilepsia corticalis sive partialis continua). Miditsinskoe Obozreni 1894;42/14:33-62.  Back to cited text no. 6
    
7.Omorokow L. Die Kojevnikoffsche Epilepsie in Sibirien. Zschr Ges Neurol Psychiat 1927;107:487-96.  Back to cited text no. 7
    
8.Proposal for revised classification of Epilepsies and Epileptic syndromes. Epilepsia 30:389-99.  Back to cited text no. 8
    
9.Sinha S, Satishchandra P. Epilepsia partialis continua over last 14 years: Experience from a tertiary care center from south India. Epilepsy Res 2007;74:55-9.  Back to cited text no. 9
    
10.Löhler J, Peters UH. Epilepsia partialis continua (Kozevnikov epilepsy). Fortschr Neurol Psychiatr Grenzgeb 1974;42:165-212.  Back to cited text no. 10
    
11.Paiboonpol S. Epilepsia partialis continua as a manifestation of hyperglycemia. J Med Assoc Thai 2005;88:759-62.  Back to cited text no. 11
    
12.Sabitha KM, Girija AS, Vargese KS. Seizures in hyperglycemic patients. J Assoc Physicians India 2001;49:723-6.  Back to cited text no. 12
    
13.Vastola EF, Maccario M, Homan R. Activation of epileptogenic foci by hyperosmolality. Neurology 1967;17:520-6  Back to cited text no. 13
    


    Figures

  [Figure 1], [Figure 2], [Figure 3]
 
 
    Tables

  [Table 1], [Table 2]

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