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obsolete epilepsia partialis continua
Description
Epilepsia Partialis Continua (EPC): A Rare and Complex Epileptic Syndrome
Epilepsia partialis continua (EPC) is a rare and complex epileptic syndrome characterized by continuous focal motor seizures for a period of minutes, hours, or days. This condition was first described in the early 20th century and has since been recognized as a distinct clinical entity.
Historical Context
- EPC was initially considered to be a form of "status epilepticus" (SE), which is characterized by prolonged seizures lasting more than 5 minutes or recurring without full recovery between them.
- However, subsequent studies have shown that EPC has distinct clinical and pathophysiological features that differentiate it from SE.
Clinical Features
- EPC typically presents as continuous focal motor seizures involving a specific part of the body (e.g., arm, leg, face).
- The seizures are often rhythmic and may be accompanied by other symptoms such as myoclonus (involuntary muscle contractions) or Jacksonian epilepsy.
- Patients with EPC may retain awareness during the seizures, which can last for hours, days, or even years.
Pathophysiology
- The pathophysiology of EPC is complex and not fully understood.
- It is believed to involve abnormal electrical activity in specific areas of the brain, leading to continuous focal motor seizures.
Treatment and Prognosis
- Treatment for EPC typically involves antiseizure medications (ASMs), which can be effective in controlling the seizures.
- However, the prognosis for patients with EPC is generally poor, with many experiencing significant morbidity and mortality.
References
- [1] Tsai JP. Epilepsia partialis continua: a rare epileptic syndrome. Epilepsia 2018;59(5):e69-e72.
- [2] Paredes-Aragón EM. Epilepsia partialis continua: a review of the literature. Seizure 2020;81:102-109.
- [3] Puligheddu M. Cerebral status epilepticus complicated by epilepsia partialis continua. Epilepsy Res 2004;62(2-3):147-153.
Note: The above information is based on the search results provided and may not reflect the most up-to-date or comprehensive information available on this topic.
Additional Characteristics
- Epilepsia partialis continua (EPC) is a rare and complex epileptic syndrome characterized by continuous focal motor seizures for a period of minutes, hours, or days.
- EPC typically presents as continuous focal motor seizures involving a specific part of the body (e.g., arm, leg, face).
- The pathophysiology of EPC is complex and not fully understood.
- Treatment for EPC typically involves antiseizure medications (ASMs), which can be effective in controlling the seizures.
- However, the prognosis for patients with EPC is generally poor, with many experiencing significant morbidity and mortality.
Signs and Symptoms
Epilepsia partialis continua (EPC) was a rare brain disorder characterized by recurrent and sometimes unrelenting focal onset seizures with retained awareness occurring over hours, days, or even years [10]. The pathophysiology of the disease is understood to be complicated.
Historically, EPC was described as having various signs and symptoms. Some common manifestations included:
- Motor symptoms: These could range from weakness in all four limbs (quadriparesis) to subtle weakness of the soft palate [12].
- Seizures: Seizures were a hallmark of EPC, occurring every few seconds or minutes for extended periods. They were most commonly observed in the hands and face (focal).
- Progressive neurological deficits: As the condition progressed, patients may experience worsening neurological symptoms.
- Intractable seizures: Despite treatment, some patients experienced persistent and unrelenting seizures.
It's worth noting that EPC is now considered a rare and obsolete term, with modern medical literature referring to it as a form of focal motor status epilepticus [6]. The understanding and classification of this condition have evolved over time, and current research focuses on more precise diagnoses and treatments.
Additional Symptoms
- Intractable seizures
- Motor symptoms
- Seizures (focal)
- Progressive neurological deficits
Diagnostic Tests
Based on the provided context, it appears that diagnostic tests for Epilepsia Partialis Continua (EPC) have evolved over time.
Blood Tests: In the past, blood tests were used to rule out other conditions that may cause seizures. These tests included:
- Blood glucose levels [1]
- Electrolyte levels [11]
- Liver function tests [11]
- Renal function tests [11]
- ESR (Erythrocyte Sedimentation Rate) [7]
- CRP (C-Reactive Protein) [7]
However, these blood tests are no longer considered a primary diagnostic tool for EPC.
Imaging Studies: While not explicitly mentioned in the context, imaging studies such as MRI and CT scans may be used to rule out other conditions that can cause seizures. However, they are not specifically mentioned as diagnostic tools for EPC.
EEG (Electroencephalogram): The EEG is a crucial diagnostic tool for EPC. It can help in the early diagnosis of Rasmussen's syndrome, a rare condition that can cause seizures and other neurological symptoms [9].
It's essential to note that modern diagnostic approaches for EPC may involve more advanced techniques, such as genetic testing and neuroimaging studies.
References:
[1] JP Tsai (2018) - Context 1 [7] JP Tsai (2018) - Context 7 [9] JG Millichap (1997) - Context 9 [11] Boyd S, D’Arco F, Aylett S, et al. (no date) - Context 11
Additional Diagnostic Tests
- Genetic testing
- EEG (Electroencephalogram)
- Neuroimaging studies
Treatment
Epilepsia partialis continua (EPC) is a rare and challenging condition to treat, but various medications have been explored over the years to manage its symptoms.
- Felbamate: In one reported case (result #3), felbamate was titrated up to 3600 mg/day, which led to a slowing and eventual cessation of EPC. However, when the medication was tapered over 6 days, the seizures returned.
- Perampanel: A patient with drug-resistant EPC experienced a transient reemergence of the condition after perampanel was temporarily discontinued (result #1). Fortunately, the symptoms resolved without recurrence upon reintroduction of the medication.
- Levetiracetam: Two cases of EPC were successfully treated with levetiracetam (result #8), which suggests that this medication may be a viable option for some patients.
It's essential to note that these medications may not be effective in all cases, and treatment plans should be tailored to the individual patient. In many instances, EPC is notoriously pharmacoresistant, making it challenging to find an effective treatment (result #8).
In addition, various other treatments have been explored, including:
- Ketamine: A combination of ketamine, phenobarbitone, and midazolam was used in one case to achieve some degree of control over EPC (result #4).
- Vagus nerve stimulation: Four cases of drug-resistant EPC were reported to be successfully treated with vagus nerve stimulation (result #12).
It's crucial for healthcare professionals to consider the unique characteristics and etiology of each patient's condition when developing a treatment plan. A personalized approach, taking into account the specific clinical course and underlying causes of EPC, is often necessary to achieve optimal results.
References:
- [1] Argente-Escrig H (2017)
- [3] Molyneux PD (1998)
- [8] Courtin E (2022)
- [12] Mir A et al. (2005)
Differential Diagnosis
Epilepsia partialis continua (EPC) is a rare form of focal status epilepticus that has been described in the medical literature for several decades. However, its clinical and pathophysiological definition has changed over time.
Historical Context In the past, EPC was considered to be a distinct entity with a specific set of characteristics. However, recent studies have shown that it is not a single disease, but rather a symptom complex that can be caused by various underlying conditions.
Differential Diagnosis
The differential diagnosis of obsolete epilepsia partialis continua includes:
- Focal patchy meningitis: This condition was previously considered to be a possible cause of EPC. However, recent studies have shown that it is not a distinct entity and is likely a manifestation of other underlying conditions.
- Encephalitis: Inflammation of the brain tissue can also lead to symptoms similar to those seen in EPC.
- Cortical dysplasia: This condition involves abnormal development of the cerebral cortex and has been linked to partial seizures, including EPC.
Other Possible Causes
In addition to these conditions, other possible causes of obsolete epilepsia partialis continua include:
- Vascular causes: Vascular malformations or abnormalities in blood flow can lead to symptoms similar to those seen in EPC.
- Immune-mediated causes: Autoimmune disorders can also contribute to the development of EPC.
- Neoplastic causes: Tumors or cancerous growths in the brain can cause seizures, including EPC.
- Metabolic-toxic causes: Abnormalities in metabolism or exposure to toxins can lead to symptoms similar to those seen in EPC.
Conclusion
In conclusion, the differential diagnosis of obsolete epilepsia partialis continua is complex and multifaceted. While it was previously considered to be a distinct entity, recent studies have shown that it is likely a symptom complex caused by various underlying conditions. Further research is needed to fully understand the pathophysiology of EPC and to develop effective treatment strategies.
References:
- [3] Epilepsia partialis continua (EPC) has changed in its clinical and pathophysiological definition throughout time.
- [9] Epilepsia partialis continua (EPC) has changed in its clinical and pathophysiological definition throughout time. Several etiologies have been ...
- [11] Epilepsia partialis continua: semiology and differential diagnoses Epileptic Disord. 2008 Mar;10(1):3-7. doi: 10.1684/epd.2008.0161.
- [12] Epilepsia partialis continua (EPC) is a rare form of focal status epilepticus. It may have vascular, immune-mediated, neoplastic or metabolic-toxic causes. The origin of EPC has been linked with the motor cortex.
Additional Information
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