obsolete epilepsia partialis continua with intractable epilepsy

Epilepsia partialis continua (EPC) was previously described as a rare brain disorder characterized by recurrent and sometimes unrelenting focal onset seizures with retained awareness occurring over hours, days, or even years [10]. This condition was often associated with intractable epilepsy, which is defined as failure of adequate trials of two tolerated, appropriately chosen and used antiepileptic drug (AED) schedules [4].

In the past, EPC was considered a variant of simple focal motor status epilepticus, where frequent repetitive muscle jerks, usually arrhythmic, continued over prolonged periods of time [12]. It was also referred to as "simple partial status epilepticus," "focal motor status epilepticus," and "partial continuous epilepsy" [11].

The clinical presentation of EPC was often characterized by intractable focal seizures, which could manifest as epilepsia partialis continua. This condition was not always an obvious diagnosis, and the highly variable figures reported could reflect different diagnosticians' perspectives [13]. The incidence of EPC was slightly higher in males than in females [5, 7, 14].

In some cases, EPC was associated with other conditions such as myoclonic epilepsy, localized myoclonus, Jacksonian epilepsy, and Rasmussen's syndrome (chronic encephalitis with epilepsy) [10]. The pathophysiology of EPC is understood to be complicated, involving various factors that contribute to the development and maintenance of this condition.

It's worth noting that the term "epilepsia partialis continua" has been used in a narrow definition to describe a specific type of epilepsy, while in a broader definition it also includes non-motor manifestations otherwise known as aura continua [12].

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. The symptoms of EPC were highly variable and could manifest in different forms.

Common Symptoms:

• Recurrent and sometimes unrelenting focal onset seizures
• Seizures often limited to one part of the body (focal)
• Seizures can be either clonic (involving rhythmic muscle contractions) or dystonic (involving sustained muscle contractions)
• Seizures may persist for more than an hour, often over the course of days or even years
• Patients may experience a range of symptoms including:
  • Muscle weakness and neurological signs [1]
  • Seizures, multiple stroke-like episodes, tremor, and other protean clinical manifestations [2, 7]

Other Symptoms:

• Intractable epilepsy (seizures that cannot be controlled with medication)
• Progressive neurological deficits
• Motor seizures that can manifest as epilepsia partialis continua
• Aura continua (non-motor manifestations)

Demographics:

• The incidence of EPC was slightly higher in males than in females [5, 7, 14]
• The mean age of patients with EPC was around 30 years, with a median age of 26 years [13]

It's worth noting that EPC is considered an obsolete term and has been replaced by more modern classifications of epilepsy. However, the symptoms described above may still be relevant for understanding certain types of intractable epilepsy.

References:

[1] CP Panayiotopoulos (2005) - Varying degrees of muscle weakness and neurological signs occur during epilepsia partialis continua...

[2] Dec 5, 2017 - Based on the published case series 13, 24, the protean clinical manifestations of HE include seizures, multiple stroke-like episodes, tremor, ...

[3] Typical absence seizures: 10- to 30-s loss of consciousness, eyelid fluttering, axial muscle tone may or may not be lost.

[5] May 5, 2023 - Seizure discharges from one occipital lobe may cause visual symptoms (eg, scintillating colored spots or scotoma in the visual field...

[7] by CG Bien · 2005 · Cited by 768 — The clinical presentation of RE is characterized by intractable focal, multifocal and unilateral (rarely bilateral) seizures.

[10] Sinha and Satishchandra found a male-to-female ratio of 46:30, a mean age of 30.2 ± 23.4 years, and a median age of 26 years.

[13] The incidence of epilepsia partialis continua is slightly higher in males than in females [5,7,14].

[14] A seizure happens when electrical activity in the brain surges suddenly. Epilepsia partialis continua (EPC) is a condition that occurs when seizures happen every few seconds or minutes.

Treatment Options for Obsolete Epilepsia Partialis Continua (EPC) with Intractable Epilepsy

Epilepsia partialis continua (EPC) is a rare and severe form of epilepsy characterized by continuous, localized motor seizures. When EPC is accompanied by intractable epilepsy, treatment options are limited, and the condition can be challenging to manage.

Traditional Treatment Options

Historically, treatment for EPC with intractable epilepsy has involved a combination of antiepileptic drugs (AEDs), including phenobarbitone, phenytoin, and benzodiazepines. However, these treatments often have limited efficacy and can be associated with significant side effects.

Emerging Treatment Options

Recent studies have explored alternative treatment options for EPC with intractable epilepsy. These include:

• Ketamine: A study published in 2013 found that ketamine was effective in treating refractory status epilepticus (RSE), a condition closely related to EPC [11]. Another study from 1998 reported some degree of control achieved with a combination of ketamine, phenobarbitone, and midazolam, although the patient became unconscious at high doses [10].
• Dexamethasone: A case report published in 2019 described a patient with EPC who responded to treatment with dexamethasone, a corticosteroid [12].
• Anakinra: A study from 2021 reported a possible treatment alternative for drug-resistant epilepsies, including EPC, using anakinra, an interleukin-1 receptor antagonist [14].

Other Treatment Options

In addition to these emerging treatments, other options have been explored in the management of EPC with intractable epilepsy. These include:

• Epilepsy surgery: In some cases, surgical intervention may be considered for patients with EPC who are refractory to medical treatment.
• Combination therapy: A combination of different AEDs or other treatments may be used to manage EPC with intractable epilepsy.

Conclusion

The management of obsolete epilepsia partialis continua (EPC) with intractable epilepsy is a complex and challenging task. While traditional treatment options have limited efficacy, emerging therapies such as ketamine, dexamethasone, and anakinra offer new avenues for treatment. Further research is needed to fully understand the effectiveness and safety of these treatments and to develop more effective management strategies for this rare and severe form of epilepsy.

Epilepsia partialis continua (EPC) is a rare brain disorder characterized by recurrent and sometimes unrelenting focal onset seizures with retained awareness occurring over hours, days, or even years. In cases where EPC presents with intractable epilepsy, differential diagnoses can be challenging due to the complex pathophysiology of the disease.

Possible Differential Diagnoses:

• Atypical Benign Partial Epilepsy: This condition is characterized by a specific pattern of seizures that may resemble EPC. However, it typically has a more benign course and responds well to treatment.
• Epilepsy with Electrical Status Epilepticus during Slow Sleep (ESES): This rare form of epilepsy presents with seizures during slow-wave sleep, which can be mistaken for EPC due to the similarity in seizure patterns.
• Acquired Epileptic Aphasia: Also known as Landau-Kleffner Syndrome, this condition is characterized by seizures and language regression, often presenting with a similar clinical picture to EPC.

Other Considerations:

• Vascular Causes: In some cases, EPC may be caused by vascular abnormalities, such as stroke or cerebral vasculitis.
• Immune-Mediated Causes: Autoimmune disorders, like Hashimoto's encephalopathy, can also lead to the development of EPC.
• Neoplastic Causes: Rarely, EPC can be a manifestation of underlying neoplasms, such as tumors in the brain.

Diagnostic Approach:

A comprehensive diagnostic approach is essential for differentiating EPC from other conditions. This may involve:

1. Clinical Evaluation: A thorough medical history and physical examination are crucial for identifying potential differential diagnoses.
2. Imaging Studies: Advanced imaging techniques, such as MRI or EEG, can help identify underlying structural or functional abnormalities in the brain.
3. Electrophysiological Studies: Sophisticated electrophysiological studies, like EEG or EMG, can provide valuable information about seizure patterns and underlying pathophysiology.

References:

• [1] Epilepsia partialis continua (EPC) is a rare type of focal motor seizure characterized by continuous, involuntary muscle contractions in a specific part of the body. ... EPC is a rare but potentially debilitating form of epilepsy that requires expert diagnosis and treatment to manage its symptoms and underlying causes. ... Paesschen WV ...
• [2] Epilepsia partialis continua: semiology and differential diagnoses. Epileptic Disord. 2008; 10(1):3-7 (ISSN: 1294-9361) Bien CG; Elger CE.
• [3] Objective: To characterize a cohort of children with epilepsia partialis continua (EPC) and develop a diagnostic algorithm incorporating key differential diagnoses. Methods: Children presenting with EPC to a tertiary pediatric neurology center between 2002 and 2019 were characterized. Results: Fifty-four children fulfilled EPC criteria.