Paraneoplastic brainstem encephalitis in a patient with malignant fibrous histiocytoma and atypical anti-neuronal antibodies

J Neurol (2004) 251 : 14151417DOI 10.1007/s00415-004-0557-2LETTER TO THE EDITORSManuel CoratoKalliope Marinou-Aktipi

Rosanna Nano

Bruno Giometto

Cristina Cereda

Guido NatoliAngelica Facoetti

Mauro CeroniParaneoplastic brainstem

encephalitis in a patient

with malignant fibrous

histiocytoma and atypical

anti-neuronal antibodiesReceived: 28 January 2004Received in revised form: 14 May 2004

Accepted: 26 May 2004Sirs: Paraneoplastic brainstem

encephalitis (BE) is often part of an

encephalomyeloneuritis, but may

also occur as an isolated clinical

entity [4]. It is mostly associated

with small cell lung cancer (SCLC),

yet some patients develop BE in the

course of other tumours. We describe a case of BE associated with

a malignant fibrous histiocytoma

(MFH) and an atypical anti-neuronal antibody.A 74-year-old man presented

with a 2 months history of myoclonus involving the upper extremities. Neurological examination

confirmed myoclonus and revealed

increased tendon reflexes on the

right. Blood studies were normal

except for a mildly increased

serum level of alfa-fetoprotein.

Brain MRI demonstrated only mild

cerebral and cerebellar atrophy.

The patient was treated with clonazepam with improvement of the

myoclonus. The search for antineuronal antibodies in the serum

revealed the presence of an atypical

antibody, raising the hypothesis of

a paraneoplastic neurological disorder (PND). Abdominal CT disclosed a retroperitoneal mass and

multiple hepatic and lymphatic

formations.Biopsy of a lateral

aortic lymph node showed a MFH.

At this time,the patients neurological state had markedly deteriorated, with dysarthria, truncal and

appendicular ataxia, right pyramidal syndrome, left hypoglossal

palsy, skew deviation, convergence

nystagmus and diplopia with paresis of the right oculomotor nerve.

Brain MRI was unchanged. Cerebrospinal fluid (CSF) examination

showed mild increase in albumin,

normal cell count and intrathecal

Ig synthesis with IgG index of 0.77

(normal upper limit for our laboratory < 0.70) and some oligoclonal

bands exclusively in the CSF. Testing for anti-neuronal antibodies

showed a 1:2000 titre in the serum,

consistent with the diagnosis of

paraneoplastic BE, and a 1:10 titre

in the CSF. The patient underwent

surgical removal of the retroperitoneal mass followed by successful

chemo- and radio-therapy, with

oncological remission and neurological improvement. Six months

later, no neurological abnormality

was present. One year after the...