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Carbamazepine hypersensitivity syndrome presenting as vasculitis of the CNS
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  1. ANTON MESEC,
  2. UROŠ ROT
  1. Department of Neurology, Medical Centre, Zaloška 7, 1105 Ljubljana, Slovenia
  2. Medical Faculty, University of Ljubljana, Korytkova 2, Ljubljana, Slovenia
  3. Department of Dermatology, Medical Centre, Ljubljana, Slovenia
  4. Department for Infectious diseases, General Hospital Celje, Oblakova 5, 3000 Celje, Slovenia
  1. Professor Anton Mesec, Department of Neurology, Medical Centre, Zaloška 7, 1105 Ljubljana, Slovenia.
  1. TATJANA PERKOVIČ
  1. Department of Neurology, Medical Centre, Zaloška 7, 1105 Ljubljana, Slovenia
  2. Medical Faculty, University of Ljubljana, Korytkova 2, Ljubljana, Slovenia
  3. Department of Dermatology, Medical Centre, Ljubljana, Slovenia
  4. Department for Infectious diseases, General Hospital Celje, Oblakova 5, 3000 Celje, Slovenia
  1. Professor Anton Mesec, Department of Neurology, Medical Centre, Zaloška 7, 1105 Ljubljana, Slovenia.
  1. TOMAŽ LUNDER
  1. Department of Neurology, Medical Centre, Zaloška 7, 1105 Ljubljana, Slovenia
  2. Medical Faculty, University of Ljubljana, Korytkova 2, Ljubljana, Slovenia
  3. Department of Dermatology, Medical Centre, Ljubljana, Slovenia
  4. Department for Infectious diseases, General Hospital Celje, Oblakova 5, 3000 Celje, Slovenia
  1. Professor Anton Mesec, Department of Neurology, Medical Centre, Zaloška 7, 1105 Ljubljana, Slovenia.
  1. BRANKO ŠIBANC
  1. Department of Neurology, Medical Centre, Zaloška 7, 1105 Ljubljana, Slovenia
  2. Medical Faculty, University of Ljubljana, Korytkova 2, Ljubljana, Slovenia
  3. Department of Dermatology, Medical Centre, Ljubljana, Slovenia
  4. Department for Infectious diseases, General Hospital Celje, Oblakova 5, 3000 Celje, Slovenia
  1. Professor Anton Mesec, Department of Neurology, Medical Centre, Zaloška 7, 1105 Ljubljana, Slovenia.

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Carbamazepine is a drug widely used in the treatment of partial and generalised tonic-clonic seizures, trigeminal neuralgia and other pain syndromes, affective disorders, and paroxysmal symptoms of multiple sclerosis. Common side effects are diplopia, dizziness, headache, nausea, and rash. Less common side effects include blood dyscrasias, toxic hepatitis, hyponatraemia as a consequence of inappropriate antidiuretic hormone secretion, orofacial dyskinesias, and cardiac arrythmias.1 Carbamazepine is also known to cause a severe systemic hypersensitivity reaction, known as carbamazepine hypersensitivity syndrome (CHS).2 It consists of a triad of fever, lymphadenopathy, and rash; so called pseudolymphoma syndrome.2 Other organs are often involved, most commonly the liver and, more rarely, lungs and kidneys.3 There are two cases of meningitis described as a complication of the carbamazepine therapy.4 5

We describe a case of severe CHS with a typical pseudolymphoma picture and involvement of other organs, including the CNS. We suggest a possible pathogenetic mechanism for encephalopathy.

A 63 year old woman was started on carbamazepine (2×200 mg), as a seizure prophylaxis after a meningeoma operation. About 3 weeks later she developed a flu-like illness. Two days afterwards, she developed a generalised rash and facial oedema, and fever appeared. She was admitted to hospital where physical examination disclosed a diffuse purpuric rash, oedema of the face and the tongue, pharyngitis, enlargement of …

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