Thalamopeduncular syndrome: Difference between revisions

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(Created page with " ## Definition & Etiology Thalamopeduncular syndrome (TPS) refers to a clinical presentation associated with lesions at the junction of the thalamus and cerebral peduncle. Primarily seen in pediatric patients (1-5% of brain tumors), it is often caused by low-grade gliomas (LGGs), such as pilocytic astrocytoma (PA) with KIAA1549-BRAF fusion or BRAF-V600E mutation. In adults, it may arise from vascular events like Artery of Percheron (AoP) occlusion leading to paramedian t...")
 
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## Definition & Etiology
Thalamopeduncular syndrome (TPS) refers to a clinical presentation associated with lesions at the junction of the thalamus and cerebral peduncle. Primarily seen in pediatric patients (1-5% of brain tumors), it is often caused by low-grade [[gliomas]] (LGGs), such as pilocytic [[astrocytoma]] (PA) with KIAA1549-BRAF fusion or BRAF-V600E mutation. In adults, it may arise from vascular events like Artery of Percheron (AoP) occlusion leading to paramedian thalamopeduncular infarction.
Thalamopeduncular syndrome (TPS) refers to a clinical presentation associated with lesions at the junction of the thalamus and cerebral peduncle. Primarily seen in pediatric patients (1-5% of brain tumors), it is often caused by low-grade [[gliomas]] (LGGs), such as pilocytic [[astrocytoma]] (PA) with KIAA1549-BRAF fusion or BRAF-V600E mutation. In adults, it may arise from vascular events like Artery of Percheron (AoP) occlusion leading to paramedian thalamopeduncular infarction.


## Clinical Presentation
== Clinical presentation ==
- **Tumoral TPS (Childhood):** Progressive spastic hemiparesis, pyramidal signs, cranial nerve (CN) palsies (e.g., oculomotor), headache, ataxia, visual impairment, focal seizures, and [[hydrocephalus]] (HCP) due to ventricular obstruction.
* '''Tumoral TPS (Childhood):''' Progressive spastic hemiparesis, pyramidal signs, cranial nerve (CN) palsies (e.g., oculomotor), headache, ataxia, visual impairment, focal seizures, and [[hydrocephalus]] (HCP) due to ventricular obstruction.
 
- **Vascular TPS (Percheron Type):** Acute decreased level of consciousness (LOC), oculomotor disturbances, ataxic gait, hemiparesis, cognitive/behavioral changes, and rarely cervical dystonia.
 
## Diagnosis
- **Imaging:** Brain MRI w/ gadolinium: Mixed solid-cystic lesions w/ heterogeneous enhancement; DTI for corticospinal tract (CST) mapping; nTMS for motor function. In vascular cases, MRI shows bilateral thalamic/midbrain infarcts.
- **Histopathology:** Confirms PA (Ki67 ~2%); molecular testing for BRAF alterations.
- **DDx:** Other thalamic/brainstem tumors, infarcts, or demyelinating diseases.


## Treatment
* '''Vascular TPS (Percheron Type):''' Acute decreased level of consciousness (LOC), oculomotor disturbances, ataxic gait, hemiparesis, cognitive/behavioral changes, and rarely cervical dystonia.
- **Surgical Management (Primary for Tumoral TPS):** Goal: Maximal safe resection. Approaches: Transsylvian (most common), transcortical transtemporal, or parietal transcortical based on tumor location & CST displacement (antero-lateral/medial/posterior). Use IONM (SSEPs, MEPs, cortical-subcortical mapping). Outcomes: GTR in ~25%, STR in ~60%, PR in ~15%; reoperation for progression.
- **Adjuvant Therapy:** Chemo (carboplatin/vincristine) or RT for residual/aggressive tumors; targeted therapy (BRAF inhibitors) if mutated.
- **Vascular TPS:** Acute stroke management (thrombolysis if indicated); no routine neurosurgical intervention unless HCP requires [[VP shunt]].


== Diagnosis ==
* '''Imaging:''' Brain MRI w/ gadolinium: Mixed solid-cystic lesions w/ heterogeneous enhancement; DTI for corticospinal tract (CST) mapping; nTMS for motor function. In vascular cases, MRI shows bilateral thalamic/midbrain infarcts.
* '''Histopathology:''' Confirms PA (Ki67 ~2%); molecular testing for BRAF alterations.
* '''DDx:''' Other thalamic/brainstem tumors, infarcts, or demyelinating diseases.


== Treatment ==
* '''Surgical Management (Primary for Tumoral TPS):''' Goal: Maximal safe resection. Approaches: Transsylvian (most common), transcortical transtemporal, or parietal transcortical based on tumor location & CST displacement (antero-lateral/medial/posterior). Use IONM (SSEPs, MEPs, cortical-subcortical mapping). Outcomes: GTR in ~25%, STR in ~60%, PR in ~15%; reoperation for progression.
* '''Adjuvant Therapy:''' Chemo (carboplatin/vincristine) or RT for residual/aggressive tumors; targeted therapy (BRAF inhibitors) if mutated.
* '''Vascular TPS:''' Acute stroke management (thrombolysis if indicated); no routine neurosurgical intervention unless HCP requires [[VP shunt]].


## Prognosis & Key Points
== Prognosis and key points ==
- **Tumoral:** Favorable w/ LGG; mean FU 5+ yrs; ~40% disease-free, ~50% stable residual; mortality <15% w/ progression. Post-op neuro deficits may improve w/ rehab.
* '''Tumoral:''' Favorable w/ LGG; mean FU 5+ yrs; ~40% disease-free, ~50% stable residual; mortality <15% w/ progression. Post-op neuro deficits may improve w/ rehab.
- **Vascular:** Variable; depends on infarct extent; potential for recovery but persistent neuro deficits common.
* '''Vascular:''' Variable; depends on infarct extent; potential for recovery but persistent neuro deficits common.
- **Neurosurgical Pearls:** Preoperative DTI/nTMS essential for CST preservation; balance resection vs. morbidity; monitor for HCP/progression.
* '''Neurosurgical Pearls:''' Preoperative DTI/nTMS essential for CST preservation; balance resection vs. morbidity; monitor for HCP/progression.


[[Category:Neuro-Oncology]]
[[Category:Neuro-Oncology]]
[[Category:Pediatric Neurosurgery]]
[[Category:Pediatric Neurosurgery]]

Latest revision as of 22:33, 27 July 2025

Thalamopeduncular syndrome (TPS) refers to a clinical presentation associated with lesions at the junction of the thalamus and cerebral peduncle. Primarily seen in pediatric patients (1-5% of brain tumors), it is often caused by low-grade gliomas (LGGs), such as pilocytic astrocytoma (PA) with KIAA1549-BRAF fusion or BRAF-V600E mutation. In adults, it may arise from vascular events like Artery of Percheron (AoP) occlusion leading to paramedian thalamopeduncular infarction.

Clinical presentation

  • Tumoral TPS (Childhood): Progressive spastic hemiparesis, pyramidal signs, cranial nerve (CN) palsies (e.g., oculomotor), headache, ataxia, visual impairment, focal seizures, and hydrocephalus (HCP) due to ventricular obstruction.
  • Vascular TPS (Percheron Type): Acute decreased level of consciousness (LOC), oculomotor disturbances, ataxic gait, hemiparesis, cognitive/behavioral changes, and rarely cervical dystonia.

Diagnosis

  • Imaging: Brain MRI w/ gadolinium: Mixed solid-cystic lesions w/ heterogeneous enhancement; DTI for corticospinal tract (CST) mapping; nTMS for motor function. In vascular cases, MRI shows bilateral thalamic/midbrain infarcts.
  • Histopathology: Confirms PA (Ki67 ~2%); molecular testing for BRAF alterations.
  • DDx: Other thalamic/brainstem tumors, infarcts, or demyelinating diseases.

Treatment

  • Surgical Management (Primary for Tumoral TPS): Goal: Maximal safe resection. Approaches: Transsylvian (most common), transcortical transtemporal, or parietal transcortical based on tumor location & CST displacement (antero-lateral/medial/posterior). Use IONM (SSEPs, MEPs, cortical-subcortical mapping). Outcomes: GTR in ~25%, STR in ~60%, PR in ~15%; reoperation for progression.
  • Adjuvant Therapy: Chemo (carboplatin/vincristine) or RT for residual/aggressive tumors; targeted therapy (BRAF inhibitors) if mutated.
  • Vascular TPS: Acute stroke management (thrombolysis if indicated); no routine neurosurgical intervention unless HCP requires VP shunt.

Prognosis and key points

  • Tumoral: Favorable w/ LGG; mean FU 5+ yrs; ~40% disease-free, ~50% stable residual; mortality <15% w/ progression. Post-op neuro deficits may improve w/ rehab.
  • Vascular: Variable; depends on infarct extent; potential for recovery but persistent neuro deficits common.
  • Neurosurgical Pearls: Preoperative DTI/nTMS essential for CST preservation; balance resection vs. morbidity; monitor for HCP/progression.
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