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Neurologic manifestation as initial presentation in a case of hereditary haemorrhagic telangiectasia.

Teo YK, Kor AC - Clin Med Insights Case Rep (2010)

Bottom Line: Hereditary Haemorrhagic Telangiectasia (HHT), or Osler-Weber-Rendu syndrome is an uncommon autosomal dominant multi-organ condition of vascular dysplasias.We describe a 19 year old Indian female who presented with cerebral abscess secondary to paradoxical emboli from pulmonary arteriovenous malformations (PAVMs) associated with HHT.Cerebral, pulmonary, hepatic and gastrointestinal involvement can be life-threatening and it is important to have lifelong follow-ups on these patients.

View Article: PubMed Central - PubMed

Affiliation: Department of Respiratory and Critical Care Medicine, Tan Tock Seng Hospital, Singapore.

ABSTRACT
Hereditary Haemorrhagic Telangiectasia (HHT), or Osler-Weber-Rendu syndrome is an uncommon autosomal dominant multi-organ condition of vascular dysplasias. We describe a 19 year old Indian female who presented with cerebral abscess secondary to paradoxical emboli from pulmonary arteriovenous malformations (PAVMs) associated with HHT. Cerebral, pulmonary, hepatic and gastrointestinal involvement can be life-threatening and it is important to have lifelong follow-ups on these patients.

No MeSH data available.


Related in: MedlinePlus

shows a 2.5 cm vague hyperdense rim-enhancing lesion (arrow) in the left thalamus with minimal mass-effect on 3rd ventricle.
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f1-ccrep-2010-015: shows a 2.5 cm vague hyperdense rim-enhancing lesion (arrow) in the left thalamus with minimal mass-effect on 3rd ventricle.

Mentions: A 19 year old Indian female with no past medical history of note was admitted for complaint of severe headache associated with fever for 5 days’ duration. On physical examination, she was alert and orientated, with a fever of 39 °C. Her neck was supple but there was a right sided paraparesis of power 4/5 with upgoing plantar reflex. Computed tomography (CT) (Fig. 1) and magnetic resonance imaging (MRI) (Fig. 2) of the brain revealed a 2 cm rim-enhancing space occupying lesion in the left thalamus with significant midline shift. The patient underwent a stereostatic biopsy of the mass and insertion of an external ventricular drain to relieve the raised intracranial pressure. Pus was removed and it showed Gram positive cocci which was later identified as streptococcus sp. She was treated with 6 weeks of intravenous ceftriaxone followed by another 6 weeks of oral amoxicillin-clavulanate. The cerebral abscess resolved with treatment and the patient gradually regained full strength of the right side of her body over the period of 3 months.


Neurologic manifestation as initial presentation in a case of hereditary haemorrhagic telangiectasia.

Teo YK, Kor AC - Clin Med Insights Case Rep (2010)

shows a 2.5 cm vague hyperdense rim-enhancing lesion (arrow) in the left thalamus with minimal mass-effect on 3rd ventricle.
© Copyright Policy - open-access
Related In: Results  -  Collection

Show All Figures
getmorefigures.php?uid=PMC3046015&req=5

f1-ccrep-2010-015: shows a 2.5 cm vague hyperdense rim-enhancing lesion (arrow) in the left thalamus with minimal mass-effect on 3rd ventricle.
Mentions: A 19 year old Indian female with no past medical history of note was admitted for complaint of severe headache associated with fever for 5 days’ duration. On physical examination, she was alert and orientated, with a fever of 39 °C. Her neck was supple but there was a right sided paraparesis of power 4/5 with upgoing plantar reflex. Computed tomography (CT) (Fig. 1) and magnetic resonance imaging (MRI) (Fig. 2) of the brain revealed a 2 cm rim-enhancing space occupying lesion in the left thalamus with significant midline shift. The patient underwent a stereostatic biopsy of the mass and insertion of an external ventricular drain to relieve the raised intracranial pressure. Pus was removed and it showed Gram positive cocci which was later identified as streptococcus sp. She was treated with 6 weeks of intravenous ceftriaxone followed by another 6 weeks of oral amoxicillin-clavulanate. The cerebral abscess resolved with treatment and the patient gradually regained full strength of the right side of her body over the period of 3 months.

Bottom Line: Hereditary Haemorrhagic Telangiectasia (HHT), or Osler-Weber-Rendu syndrome is an uncommon autosomal dominant multi-organ condition of vascular dysplasias.We describe a 19 year old Indian female who presented with cerebral abscess secondary to paradoxical emboli from pulmonary arteriovenous malformations (PAVMs) associated with HHT.Cerebral, pulmonary, hepatic and gastrointestinal involvement can be life-threatening and it is important to have lifelong follow-ups on these patients.

View Article: PubMed Central - PubMed

Affiliation: Department of Respiratory and Critical Care Medicine, Tan Tock Seng Hospital, Singapore.

ABSTRACT
Hereditary Haemorrhagic Telangiectasia (HHT), or Osler-Weber-Rendu syndrome is an uncommon autosomal dominant multi-organ condition of vascular dysplasias. We describe a 19 year old Indian female who presented with cerebral abscess secondary to paradoxical emboli from pulmonary arteriovenous malformations (PAVMs) associated with HHT. Cerebral, pulmonary, hepatic and gastrointestinal involvement can be life-threatening and it is important to have lifelong follow-ups on these patients.

No MeSH data available.


Related in: MedlinePlus