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unilateral focal polymicrogyria

Description

Unilateral Focal Polymicrogyria (BFPP) Description

Unilateral focal polymicrogyria (BFPP) is the mildest subtype of polymicrogyria, a cerebral cortical malformation characterized by excessive folding and abnormal layering of the brain's surface. This condition affects a relatively small area on one side of the brain, typically involving the cortex surrounding the sylvian fissures in approximately 80% of cases [5].

The symptoms and characteristics of BFPP include:

  • Mild cortical malformation: The mildest form of polymicrogyria, with excessive folding and abnormal layering of the cerebral cortex.
  • Unilateral involvement: Affects a relatively small area on one side of the brain.
  • Frontal lobe involvement: Typically involves the frontal lobe, particularly the cortex surrounding the sylvian fissures in approximately 80% of cases [5].
  • Variable severity: Can be associated with contralateral hemiparesis (weakness or paralysis on the opposite side of the body), developmental delay, and focal seizures [7].

Overall, unilateral focal polymicrogyria is a rare and mild form of polymicrogyria, characterized by excessive cortical folding and abnormal layering in a small area of the brain.

Additional Characteristics

  • Unilateral focal polymicrogyria (BFPP) is the mildest subtype of polymicrogyria.
  • Affects a relatively small area on one side of the brain, typically involving the cortex surrounding the sylvian fissures in approximately 80% of cases.
  • Characterized by excessive folding and abnormal layering of the cerebral cortex.
  • Can be associated with contralateral hemiparesis, developmental delay, and focal seizures

Signs and Symptoms

Unilateral focal polymicrogyria (BFPP) is a type of cerebral cortical malformation characterized by an abnormal increase in the number of small gyri on one side of the brain. The signs and symptoms of BFPP can vary, but they often include:

  • Recurrent seizures (epilepsy): This is a common symptom of BFPP, with seizures occurring due to the abnormal brain tissue [1][3].
  • Delayed development: Children with BFPP may experience delayed development in areas such as speech, language, and cognitive skills [4][6].
  • Crossed eyes: Some individuals with BFPP may have crossed eyes or other visual problems [2][5].
  • Problems with speech and swallowing: Speech and language difficulties are common in individuals with BFPP, and some may also experience problems with swallowing [1][7].

It's worth noting that the severity of these symptoms can vary depending on the extent and location of the polymicrogyria. In general, unilateral focal polymicrogyria tends to cause milder symptoms compared to bilateral forms of the condition.

References: [1] Context result 6 [2] Context result 5 [3] Context result 7 [4] Context result 8 [5] Context result 9 [6] Context result 1 [7] Context result 4

Additional Symptoms

  • Delayed development
  • Recurrent seizures (epilepsy)
  • Crossed eyes
  • Problems with speech and swallowing

Diagnostic Tests

Unilateral focal polymicrogyria (BFPP) is a type of cerebral cortical malformation characterized by excessive cortical folding, typically affecting a small area on one side of the brain.

Imaging Studies

The diagnostic tests for unilateral focal polymicrogyria include:

  • High-resolution MRI: This is considered the most reliable imaging modality for diagnosing and evaluating the distribution and extent of the dysgenesis in BFPP. Thin slice volumetric T1-weighted images are particularly useful in assessing the detail and associated structural abnormalities [7].
  • MRI with thin slice volumetric T1-weighted images: This should be included as part of the imaging protocol to ensure accurate diagnosis and evaluation of the condition [7].

Other Diagnostic Considerations

While not directly related to diagnostic tests, it's worth noting that:

  • The mildest form of polymicrogyria is known as unilateral focal polymicrogyria (BFPP), which affects a relatively small area on one side of the brain. This form may cause mild symptoms or be asymptomatic [8][9].
  • Genetic testing in PMG has a low diagnostic yield, ranging from 12 to 20% in different studies [3].

References:

[1] by S Kolbjer · 2023 · Cited by 5 — [7] by RJ Leventer · 2010 · Cited by 285 — [8] Unilateral focal polymicrogyria (BFPP) is the mildest sub-type of polymicrogyria (PMG), a cerebral cortical malformation characterized by excessive cortical folding. [9] The mildest form is known as unilateral focal polymicrogyria. This form of the condition affects a relatively small area on one side of the brain. It may cause mild symptoms or be asymptomatic.

Additional Diagnostic Tests

  • High-resolution MRI
  • MRI with thin slice volumetric T1-weighted images

Treatment

Treatment Options for Unilateral Focal Polymicrogyria

Unilateral focal polymicrogyria (UFP) is a rare brain condition characterized by excessive cortical folding, which can lead to seizures and other neurological problems. While there is no specific treatment to get rid of this condition, various drug treatments are available to manage its symptoms.

Anti-Seizure Medications

The first-line treatment for persons with seizures associated with UFP is anti-seizure medications (1). The medication chosen depends on the type of seizures or epilepsy syndrome. These medications can help control mild seizures that may be easily controlled with medication (5, 10).

Surgical Treatment

In some cases, surgical treatment may be considered to remove the affected area of the brain. A few case reports have described successful surgical treatment of patients with focal PMG by resection of epileptic foci (3). However, this option is typically reserved for severe epilepsy or when other treatments have failed.

Current Research and Recommendations

Recent studies suggest that surgical treatment may be superior to nonsurgical management in the treatment of drug-resistant epilepsy (DRE) associated with UFP (8). Complete resection of the anatomical PMG can lead to significant improvements in seizure control. However, more research is needed to fully understand the benefits and risks of surgical treatment for UFP.

Conclusion

While there is no specific treatment to get rid of unilateral focal polymicrogyria, various drug treatments are available to manage its symptoms. Anti-seizure medications are often the first line of treatment, while surgical treatment may be considered in severe cases or when other treatments have failed. Further research is needed to fully understand the best treatment options for UFP.

References: [1] [2] [5] [6] [10] Note: The numbers in square brackets refer to the context search results used to generate this answer.

Recommended Medications

  • Surgical Treatment
  • Anti-Seizure Medications

💊 Drug information is sourced from ChEBI (Chemical Entities of Biological Interest) database. Always consult with a healthcare professional before starting any medication. Click on any medication name for detailed information.

Differential Diagnosis

Unilateral Focal Polymicrogyria Differential Diagnosis

Polymicrogyria, a rare brain malformation, can manifest in various forms, including unilateral focal polymicrogyria (UFP). The differential diagnosis for UFP involves considering other conditions that may present with similar symptoms and characteristics. Here are some possible differential diagnoses:

  • Gangliogliomas: These are low-grade tumors that can cause seizures, developmental delay, and cognitive impairment, similar to UFP.
  • Focal cortical dysplasia: This condition is characterized by abnormal brain tissue in a specific area of the cortex, which can lead to seizures, hemiparesis, and other neurological symptoms.
  • Unilateral polymicrogyria: As mentioned earlier, this condition involves excessive cortical folding on one side of the brain, which can cause similar symptoms to UFP.

Key Features to Consider

When differentiating between these conditions, consider the following key features:

  • Location and extent of brain involvement: The location and extent of brain involvement can help distinguish between UFP and other conditions.
  • Symptom severity and progression: The severity and progression of symptoms can also provide clues about the underlying condition.
  • Imaging findings: Imaging studies such as MRI or CT scans can help identify characteristic features of each condition.

References

  • [4] Unilateral polymicrogyria may be seen in various cortical locations, but manifesting signs and symptoms typically include spastic hemiparesis primarily of the upper extremity, variable degrees of mental retardation, and seizures.
  • [5] Polymicrogyria is a common malformation of cortical development, where there is abnormal layering, excessive gyration (folding), and gyral fusion in the cerebral cortex.
  • [12] Unilateral polymicrogyria is a cerebral cortical malformation characterized by unilateral excessive cortical folding and abnormal cortical layering.

Note: The above information is based on the search results provided, which include descriptions of various conditions related to polymicrogyria.

Additional Differential Diagnoses

  • Gangliogliomas
  • Focal Cortical Dysplasia
  • Unilateral Polymicrogyria

Additional Information

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