Cerebral Cavernous Malformation Classification [Neurosurgery Wiki]

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====== Cerebral Cavernous Malformation Classification ======

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Cerebral cavernous malformations (CCMs) can be classified based on histopathology, genetics, radiological features, clinical presentation, and anatomical location.

===== 1. Histopathological Classification =====
CCMs are vascular lesions composed of clusters of dilated, thin-walled capillaries without intervening brain parenchyma. They lack normal vessel structure, with no smooth muscle or elastic tissue.

==== Zabramski Classification (1994) ====
see [[Zabramski Classification]]



===== 2. Genetic Classification =====
CCMs can be **sporadic** or **familial (autosomal dominant with incomplete penetrance)**.

  * **Sporadic CCMs**: Usually solitary, often associated with developmental venous anomalies (DVAs).
  * **Familial CCMs**: Multiple lesions with a higher risk of recurrence due to genetic mutations.

see [[Familial cerebral cavernous malformation]].

Sporadic CCMs occur in people with no family history of the disorder. These individuals tend to have only one CCM.
Those with sporadic CCM do not have a greater chance of having a child with a CCM than anyone else in the general population.

Three main genes are implicated:

  * **CCM1 (KRIT1)** – Chromosome 7q21.2
  * **CCM2 (MGC4607)** – Chromosome 7p15-13
  * **CCM3 (PDCD10)** – Chromosome 3q26.1

===== 3. Clinical Classification =====
  * **Asymptomatic**: Incidental findings on imaging.
  * **Symptomatic**:
    - **Seizures**: Most common presentation.
    - **Hemorrhagic**: Acute or chronic bleeding leading to focal neurological deficits.
    - **Progressive neurological deterioration**: Due to recurrent hemorrhages or mass effect.

===== 4. Location-based Classification =====
  * **Supratentorial**: Commonly associated with seizures.
  * **Infratentorial**: Can lead to brainstem dysfunction.


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This classification system helps in assessing prognosis, determining treatment strategies, and understanding genetic risks.



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see [[Insular Cavernous Malformation]]

see [[Frontal lobe cavernous malformation]].

see [[Temporal lobe cavernous malformation]].