Epilepsia partialis contina (EPC) in a narrow definition is a variant of simple focal motor status Epilepsia partialis continua (EPC) was first described by. •EPC has many possible local or systemic etiologies. •Motor and non-motor variants exist. •Four characteristic different time courses can be. Epilepsia partialis continua (EPC) is a rare type of localization-related motor epilepsy. Clinical spectrum, electroencephalo- graphy (EEG) characteristics and .
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Epilepsia partialis continua (EPC) is a rare brain disorder where a patient experiences recurrent and, sometimes, unrelenting focal onset. Epilepsia partialis continua (EPC) was first described by Kozhevnikov in a report on four patients with “a special form of cortical epilepsy” to the Russian Society. Summary. Epilepsia partialis continua (EPC) is defined clinically as a syndrome of continuous focal jerking of a body part, usually localized to a distal limb.
Some evidence has also shown Jacksonian spread and secondary generalization of seizures, highlighting the complex pathophysiology of the disease. Neuronal cell loss, astrogliosis, and blood-brain barrier damage brought on by these cytokines may contribute to the underlying hyperexcitability of the cortex.
Epilepsia partialis continua
Classically, EPC presents as a focal form of status epilepticus SE with a preserved consciousness that is restricted to one part of the body, occurring for one hour or more, recurrent, and associated with postictal confusion and weakness.
Motor deficits ranging from quadriparesis to subtle soft palate paresis may be noted.
Sensory deficits, similarly, are variable but are less common compared to their motor counterparts. The involvement of the upper extremities is more common. Imaging MRI findings can help in delineating structural lesions.
Focal cortical dysplasia and Rasmussen encephalitis, two common causes of EPC, are more likely to be identified on MRI because of the advancements in imaging quality and technology in recent years. Standard imaging modalities used during neurological workups, such as computed tomography CT and MRI scanning, may show no pathology. Sometimes, asymmetrical background slowing is noted.
Studies using SSEPs have shown hyperexcitability of the motor cortex and the subsequent exaggerated responses to peripheral stimuli. The central aspect of any treatment regimen is seizure control using anti-epileptic medication AED. Some drugs have been seen to be more effective than others, but polytherapy using multiple AEDs is fairly common.
Studies have shown that phenytoin and phenobarbital may be more effective than valproate or carbamazepine. Parents are second degree cousins. Her mother is a year-old housewife. Her father is a year-old soldier.
Both parents finished high school education, There is no history of recurrent abortions. There is no history of similar cases. They are living in Assir region, Saudi Arabia, with fair socioeconomic status.
They have a 4-year-old normal healthy boy. During pregnancy, mother noticed decreased fetal movements: with no polyhydramnios, no oligohydramnios.
She was born full term through spontaneous vertex vaginal delivery with normal birth weight and head circumference. The mother and the child were discharged home next day after delivery in good condition. She had multiple hospital admissions before she presented to our hospital for abnormal continuous rhythmic hand movements. At the age of 14 months, she started to develop sudden onset of continuous right hand movements, where they went to a local hospital and her EEG was abnormal and was given intravenous medications without benefit.
Then she was extubated and transferred to the ward. She was discharged home unable to sit or hold things with hands and unable to speak. She was put on topiramate, phenobarbitone, and clonazepam. At home, her mother did not give the antiepileptic medications. Seizures recurred again with the same semiology plus right mouth twitches and occasional deviation of both eyes to the right side. The parents tried traditional treatment without benefit.
They went back to local hospital and underwent the same course of treatment. She was shifted to another hospital and was investigated with lab works, EEG, and brain MRI and the parents were informed of normal results. After 2 months, she started to have decreased oral intake, increased sputum secretion, and bluish discoloration of lips and face with no abnormal movements. She had an EEG that was abnormal.
She was admitted to the PICU for 2 days. Her EEG was normalized and she stopped seizing. She was discharged home on the aforementioned three antiepileptic medications. One month later, she started to have decreased feeding, perioral bluish discoloration, and unresponsiveness.
She was admitted to the hospital: needing escalating doses of her medications. She became unable to follow objects with her eyes and her right hand movements were increasing in intensity during awake time. They were described as rhythmic, continuous, not stopping when held, and lasting for 2 weeks. They were not associated with bluish discoloration or mouth deviation. The parents mentioned that she had constipation and episodes of hypothermia.
They mentioned that she was able to transfer objects at the age of 7 months, waved bye-bye at 9 months, sat with support at 10 months, and used to say mama, baba, nanna milk , and dada grandma. She was continued on the same antiepileptic medications. Examination when she presented to us revealed smooth straight black colored hair, right upper limb rhythmic, and continuous movements. Weight was 8 kg below 3rd centile, length was 77 cm on the 25th centile, and head circumference was 45 cm between the 10th and 25th centiles.
She was not fixing and not following. She had preserved light reflex with bilateral equal reactive pupils. Fundoscopy was normal. There was no facial asymmetry, and gag reflex was preserved. She was responding to sounds. She was not able to move her right upper and lower limbs. There were no fasciculations, but decreased axial tone, with appendicular hypotonia on the right more than the left side. Spine, skin, and other systemic examinations were normal. Her investigations included tandem mass spectrometry, urine gas chromatograph, erythrocyte sedimentation rate, copper, zinc, copper, 24 h urine, ceruloplasmin, uric acid, very long chain fatty acids, C3,C4, serum ammonia, serum lactate, plasma amino acids, and cerebrospinal fluid CSF glucose, protein, Herpes Simplex virus, Epstein Barr virus, Cytomegalovirus, amino acids, ammonia, lactate.
They were all within normal. Her electroencephalogram EEG showed excessive fast activity in the beta range over the posterior head regions and sharp waves at left temporoparietal region. The sharp waves were not time locked with the right hand rhythmic activity. Rare sharp transients and sharp waves were seen independently at right temporal at T8. There were prolonged episodes of the right hand rhythmic clonic activity that occurred episodically and lasted anywhere from 42 minutes to almost 1 hour.
The patient had these events while awake as well as during sleep but with less intensity. There were no changes in her level of consciousness or her vital signs during these events.
THE DANCE OF DOOM A RARE CASE OF EPILEPSIA PARTIALIS CONTINUA
The events did not involve any other body parts apart from the right hand. Brain magnetic resonance imaging MRI showed cortical and cerebellar atrophy, right-sided subdural collection noted over the right frontal and parietal convexity Figures 1 a and 1 b ; however, there was no associated compression effect noted over the underling cortical sulci.
White matter T2 hyperintensity involving the centrum semiovale and the occipital lobes Figures 2 a and 2 b. Evidence of right frontal cortically located lesion showing diffusion restriction indicating cytotoxic edema due to ischemia.
Interval development of bilateral and symmetrical basal ganglia T2 hyperintensities affecting the putamina and thalami as well as the dentate nuclei. Figure 1: Brain magnetic resonance imaging MRI showed cortical and cerebellar atrophy, right-sided subdural collection noted over the right frontal and parietal convexity. Figure 2: White matter T2 hyperintensity involving the centrum semiovale and the occipital lobes.
Diffuse tensor imaging DTI showed reduction of the diffusion anisotropy noted in the left occipital cortical lesion with reduced white matter thickness of the sagittal stratum. Magnetic resonance spectroscopy MRS obtained at the level of internal capsule and at the parietal white matter demonstrated reduced N-acetyl aspartate peak at the parietal white matter indicating neuronal loss with low choline peak at the internal capsule Figures 4 and 5.
Figure 4: Magnetic resonance spectroscopy MRS obtained at the level of internal capsule demonstrated low choline peak. Figure 5: Magnetic resonance spectroscopy MRS obtained at the level of the parietal white matter demonstrated reduced N-acetyl aspartate peak at indicating neuronal loss. Echocardiogram done on showed small ASD secundum. Hair biopsy done on showed that most of the hair shafts examined under light microscopy were unremarkable. Revealed the presence of a heterozygous mutation, C.
Discussion Menkes disease is a rare X-linked recessive neurodegenerative disorder, nearly exclusively affecting males. Affected infants are healthy at birth and have early normal development for the first 2—4 months and then recognition of seizures and hypotonia and failure to thrive begin and they often die by the age of 3 years [ 6 ].
They have characteristic hair that is short, scanty, and hypopigmented [ 6 ]. The hair is even shorter on the sides and the back of the head than on the top. The twisted strands may be reminiscent of those in steel wool cleaning pads. The eyebrows usually share the unusual appearance. Light microscopy of patient hair illustrates pathognomonic pili torti i.
Hair may demonstrate unusual colors, such as white, silver, or grey; however, in some individuals with Menkes disease, the hair is pigmented normally. The typical patients have jowly faces, with sagging cheeks and ears that often appear large.
They have high-arched palates, with delayed teeth eruption [ 7 ]. Profound truncal hypotonia is invariably present. This may be accompanied with increased appendicular tone. Deep tendon reflexes are often hyperactive. The suck and cry are usually strong [ 8 ].
Visual fixation and tracking commonly are impaired [ 9 ]. Epilepsy is found to be a major feature where seizures are usually of focal pattern which may progress to epileptic spasms in the early stages and as tonic seizures and myoclonic jerks in the late stages [ 10 ].
Neuroimaging studies show atrophy of grey matter, ventriculomegaly, and tortuous intracranial vasculature with white matter signal changes consistent with loss of myelin and axons [ 12 ].No relevant family history.
Apr ; 74 1 : She was eventually found in bed not responding verbally. Ethical Approval No ethical approval was required. Genetic background determines such features as height, eye color, and potential to develop certain diseases like diabetes, but it also determines all the chemicals and structures that make up the brain, therefore playing a role in Epilepsia partialis continua.
Intravenously administered valproate, diazepam and thiopental sodium are more successful, however at the cost of adverse effects. Microarray studies suggest widespread effects in dysregulation of genes involved in cellular responses to oxidative stress, ribosomal translation, signal transduction, mitochondrial function, and immune responses. Weight was 8 kg below 3rd centile, length was 77 cm on the 25th centile, and head circumference was 45 cm between the 10th and 25th centiles.
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