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Year : 2001  |  Volume : 49  |  Issue : 3  |  Page : 287-90

Febrile status epilepticus as the first presentation of cortical developmental malformation : report of 2 cases.

Department of Internal Medicine, Dayanand Medical College, Ludhiana, Punjab-141 001, India.

Correspondence Address:
Department of Internal Medicine, Dayanand Medical College, Ludhiana, Punjab-141 001, India.
[email protected]

  »  Abstract

Cortical developmental malformations are an important cause for epilepsy. The epileptic disorder usually begins with sporadic seizures, followed by gradually increasing seizure frequency and culminating in epilepsia partialis continua. We report the de-novo occurrence of focal status epilepticus accompanying hyperpyrexia in two male children, aged 18 months and 4 years. One child had prior neuro-developmental delay, while in the other, developmental milestones were normal. In both instances, there were no seizures prior to the presenting neurological illness. Cerebrospinal fluid examination was normal in both the cases. Magnetic resonance imaging revealed extensive pachygyria in one patient and focal cortical thickening in the other. Therefore, cortical developmental malformation may be suspected as an underlying aetiology in children presenting with de-novo status epilepticus and hyperpyrexia.

How to cite this article:
Singh G, Dhanuka A K, Bains H S, Singh D. Febrile status epilepticus as the first presentation of cortical developmental malformation : report of 2 cases. Neurol India 2001;49:287

How to cite this URL:
Singh G, Dhanuka A K, Bains H S, Singh D. Febrile status epilepticus as the first presentation of cortical developmental malformation : report of 2 cases. Neurol India [serial online] 2001 [cited 2023 Dec 11];49:287. Available from:

   »   Introduction Top

Cortical developmental malformations are being increasingly recognised as a cause for epilepsy.[1],[2],[3] The recent widespread use of magnetic resonance imaging (MRI) in the study of epilepsy has lead to improved diagnosis and understanding of these mal-formations.[4],[5] A large variety of malformations have been reported in the literature.[6],[7],[8],[9] Several authors have proposed classifications of these malformations based upon MR morphology, patterns of inheritance and clinical presentations.[2],[10] A variety of seizure types and frequencies are known to occur in persons with cortical developmental malformations.[2],[3],[11],[13] We report two children with cortical developmental malformations, in whom the presenting epileptological syndrome was life threatening focal status epilepticus. To the best of our knowledge, this is the first report of such presentation of malformations of cortical development.

   »   Case report Top

Case 1 : PS, a four and a half year old boy, was admitted with fever and seizures with loss of
consciousness of 5 and 2 days duration, respectively. Seizures were typically left focal, occurred hourly, and were prolonged. The child remained unconscious in between the seizures. He had normal development and had no seizures prior to this. Height, weight and head circumference were within normal limits. Investigations revealed normal haemogram and serum biochemistry. Two blood smears for malarial parasite were negative. Blood cultures, urine cultures and Widal were negative. Lumbar CSF examination was essentially normal. MR examination revealed focal cortical malformation with abnormally thickened cortex in the right parietal lobe [Figure - 1]. EEG disclosed bilateral slow waves (theta to delta range), and absence of fast beta activity on the right side. A presumptive diagnosis of acute viral encephalitis was made. The child was dilantinised (18 mg/Kg, over 20 minutes). Since seizures persisted, a loading dose of phenobarbitone (15 mg/Kg body weight) was administered. Subsequently, the child was intubated and administered sodium thiopentone, 5 mg/Kg bolus, followed by infusion at the rate of 0.2 mg/Kg/hour. Seizures were partially controlled within 4 hours of admission. Daily maintenance doses of phenytoin, phenobarbitone and clobazam were instituted. Thiopentone infusion was tapered off between the 3rd and 5th days. The child continued to have breakthrough seizures for the first 7 days of admission, but was subsequently seizure free. He was discharged on maintenance doses of phenytoin sodium (5mg/Kg/day), phenobarbitone (5mg/Kg/day) and clobazam (5 mg twice a day).
Case 2 : SK, a one and half year old boy was admitted with fever and approximately 20 seizures over 24 hours. Fever was high grade (104-106oF) and preceded the first seizure by 6-8 hours. Initial seizures were generalised tonic-clonic. However, while in hospital, the child had continuous focal tonic seizures involving the right upper and lower extremities. He retained partial consciousness in between seizures. The child was born of a domiciliary delivery. Motor milestones were delayed and he had not started walking till the onset of the present acute neurological event. In addition, he was known to be left-handed. On specific inquiries, the prior occurrence of subtle focal seizures and motor deficit involving the right upper and lower limbs were denied. Initial investigations revealed moderate mixed deficiency anaemia (Hb: 9g/dl). Serum biochemistry including electrolytes, liver functions, serum Ca++ and Mg++ were within normal limits. Blood film for malarial parasite, Widal, blood cultures and urine cultures were noncontributory. Lumbar puncture revealed normal opening pressure WBCs - 2/hpf, protein - 28 mg/dl and sugar - 56 mg/dl. IgM and IgG antibody titers for Herpes simplex virus were within normal range. A loading dose of phenytoin sodium (15 mg/Kg) was given. Since seizures persisted, phenobarbitone (20 mg/Kg in 100 ml of normal saline infused over 20 minutes) was also administered. There were no further seizures and daily maintenance doses of phenytoin and phenobarbitone were continued during and after hospitalisation. Fever subsided within 7-8 days of hospitalisation.
MRI revealed disclosed marked focal cortical thickening, poorly formed gyri and shallow sulci in the left frontal and parietal lobes. In addition, there was loss of cortical signal over the left parietooccipital region with relative loss of gray-white matter interface in the same region [Figure - 2]. Electroencephalographic examination, performed two days after control of seizures, and repeated prior to discharge, revealed slow wave bihemispherical dysfunction and was remarkable for reduced amplitude and absence of fast rhythms on the left side [Figure - 3].

   »   Discussion Top

Malformations of cortical development are usually associated with intractable partial epilepsy. However, a variety of epileptic syndromes and seizure types have been reported to occur with developmental malformations. In a report of a large series of 100 adult patients, Raymond et al found that 86% of cases had localisation related epilepsy, only 14% cases had generalised epilepsy.[2] A number of developmental malformations including focal pachygyria and microgyria, focal cortical dysplasia and heterotopias occur predominantly in the frontal and temporal lobes.[2],[3] Understandably, therefore, frontal and temporal lobe epilepsies are the commonest type of localisation related epilepsy in persons with developmental malformations.[2],[3]
While symptomatic localisation related epilepsy remains the commonest epileptological manifestation of cortical developmental malformations, other seizure types may also be observed. Palmini et al reported tonic or atonic drop attacks in 27% patients in a series of 30 patients with focal neuronal migration disorders.[3] Other previously described presentations include focal cortical myoclonus due to dysplasia of the rolandic cortex and epilepsia partialis continua.[11],[12],[13] Kuzniecky and Powers, reported one patient with histlogically proven focal cortical dysplasia, who initially developed focal seizures and later; seizures increased in frequency, ultimately leading to epilepsia partialis continua and progressive focal neurological deficit.[12] In addition, the patient had neuropsycho-logical impairment and diffuse bihemispherical electroencephalographic abnormalities, features that identify with Bancaud's group II, epilepsia partialis continua.[14] The authors concluded that, besides Rasmussen's encephalitis, cortical dysplasia is an important cause for group II, epilepsia partialis continua.[15]
The course of epilepsy due to cortical developmental malformations has been described by several authors.[2],[3],[11],[12],[13] The onset of first seizure is between early infancy and 20 years of age, though a typical patient would develop the first seizure by about 5 years of age. Seizures are initially sporadic but their frequency rapidly increases over a period of 1-3 years, to weekly or monthly seizures. At this point of time, focal or generalised status epilepticus or more characteristically, epilepsia partialis continua can occur. Kuzniecky et al reported 4 cases in whom the initial manifestation was focal cortical myoclonus.[11] These individuals ultimately developed epilepsia partialis continua, 1-4 years after the onset of first seizure. Desbiens et al reported 4 patients with occult cortical dysplasia, who had focal status epilepticus 1836 months after the first seizure.[16] In several of the larger series of patients with cortical developmental malformations, status epilepticus occurred in 15-30% of cases.[2],[3] However, these reports do not mention the time of onset of status epilepticus relative to first seizure.
Upon a thorough literature search, we could not identify a single previously reported instance, where status epilepticus was the first manifestation of a cortical developmental malformation. Infact, the association of status epilepticus with a febrile illness, lead us to consider an encephalitic illness as a likely cause of seizures. However, MRI disclosed the presence of a cortical dysplasia in both children. Hyperpyrexia has been reported to precipita status epilepticus in 2 patients with highly epileptogenic developmental malformations.[17]
Focal rolandic seizures can be subtle. It may be argued that, the families of the two children under discussion did not notice subtle focal seizures prior to the present episodes of status epilepticus. However, specific intensive inquiries did not disclose historical evidence of subtle seizures. Therefore, the unique clinical presentation and the underlying cytoarcheitectonic abnormalities identified upon MR can be knitted into a new syndromic presentation of cortical developmental malformations. The latter, in turn, may be added to the list of causes of de-novo febrile status epilepticus, which include, encephalitis, meningitis, brain abscess, subdural empyema, septic venous thrombosis, cerebral malaria and enteric encephalopathy.[18],[19],[20]


  »   References Top

1.Taylor DC, Falconer MA, Bruton CJ et al : Focal dysplasia of the cerebral cortex in epilepsy. J Neurol Neurosurg Psychiatry1971; 34 : 369-387.   Back to cited text no. 1    
2.Raymond A, Fish DR, Sisidiya SM et al : Abnormalities of gyration, heterotopias, tuberous sclerosis, focal cortical dysplasia, microdysgenesis, dysembroplastic neuroepithelial tumour and dysgenesis of the archicortex in epilepsy. Clinical, EEG and neuroimaging features in 100 adult patients. Brain1995; 118 : 629-660.   Back to cited text no. 2    
3.Palmini A, Andermann F, Oliver A et al : Focal neuronal migration disorders and intractable epilepsy : a study of 30 patients. Ann Neurol 1991; 30 : 741-749.   Back to cited text no. 3    
4.Barkovich AJ, Gressens P, Evrard P : Formation, maturation and disorders of brain neocortex. AJNR 1992; 13 : 423-446.   Back to cited text no. 4    
5.Palmini A, Andermann F, Oliver A et al : Neuronal migration disorders: a contribution of modern neuroimaging to the diagnosis of epilepsy. Can J Neurol Sci 1991; 18 : 580-587.   Back to cited text no. 5    
6.Barkovich AJ, Kjos BO : Schizencephaly: correlation of clinical findings with MR characteristics. AJNR 1992; 13 : 104-106.   Back to cited text no. 6    
7.Barkovich AJ, Jackson JE Jr, Boyer RS : Band heterotopias: a newly recognized neuronal migration anomaly. Radiology 1989; 171 : 455-458.   Back to cited text no. 7    
8.Barkovich AJ, Kjos BO : Gray matter heterotopias: MR characteristics and correlation with developmental and neurologic manifestations. Radiology1992; 182 : 493-499.   Back to cited text no. 8    
9.Barkovich AJ, Kjos BO : Nonlissencephalic cortical dysplasias: correlation of imaging findings with clinical deficits. AJNR1992; 13 : 95-103.   Back to cited text no. 9    
10.Barkovich AJ, Kuzniecky RI, Dobyns WB et al : A classification scheme for malformations of cortical development. Neuropediatrics1996; 27 : 59-63.   Back to cited text no. 10    
11.Kuzniecky R, Berkovic S, Andermann F et al : Focal cortical myoclonus and rolandic cortical dysplasia: clarification by magnetic resonance imaging. Ann Neurol 1988; 23 : 317-325.   Back to cited text no. 11    
12.Kuzniecky R, Powers R : Epilepsia partialis continua due to cortical dysplasia. J Child Neurol 1993; 8 : 386-388.   Back to cited text no. 12    
13.Fusco L, Bertini E, Vigevano F : Epilepsia partialis continua and neuronal migration anomalies. Brain Dev1992; 14 : 323-328.   Back to cited text no. 13    
14.Bancaud J : Kojewnikow's syndrome (epilepsia partialis continua) in children. In : Epileptic syndromes in infancy, childhood and adolescence. Roger J, Bureau M, Dravet Ch (Eds.), 2nd ed. John Libbey, London. 1992; 363-379.   Back to cited text no. 14    
15.Andermann F : Chronic encephalitis and epilepsy: Rasmussen's syndrome. Butterworth Heinemann, Boston. 1991.   Back to cited text no. 15    
16.Desbiens R, Berkovic SF, Dubeau F et al : Life threatening focal status epilepticus due to occult cortical dysplasia. Arch Neurol 1993; 50 : 695-700.   Back to cited text no. 16    
17.Spreafico R, Battaglia G, Arcelli P et al : Cortical dysplasia : an immunocytochemical study of three patients. Neurology 1998; 50 : 27-36.   Back to cited text no. 17    
18.Hauser WA : Status epilepticus: etiology and neurological sequelae. Adv Neurol1983; 34 : 3-14.   Back to cited text no. 18    
19.Aicardi J, Chevrie JJ : Convulsive status epilepticus in infants and children: a study of 239 cases. Epilepsia1970; 11 : 187-197.   Back to cited text no. 19    
20.Barry E, Hauser WA : Status epilepticus : the interaction of epilepsy and acute brain disease. Neurology 1993; 43 : 1473-1478.   Back to cited text no. 20    


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