healthmedicinet

A 61 year-old, Caucasian man, with controlled type II diabetes, came to our attention
in April 2011 because of loss of balance that progressed over weeks. There was no
family history of neurological or autoimmune disorders. In the preceding month, he
started noticing body imbalance, reduced ability to focus on daily activities, to
elaborate thoughts, and incoordination. Neurological examination revealed signs associated
with acute cerebellar degeneration, such as dysdiadochokinesia, mild dysarthria, dizziness,
vertigo and clear ataxia. Baseline International Cooperative Ataxia Rating Scale (ICARS
score) 4] was 18. Insulin, gastrin, glucagon, C-peptide, thyroid stimulating hormone, thyroxine,
folic acid, vitamin B-12 serum levels and urinary 5-hydroxyindoleacetic acid (5HIAA)
levels were normal. Results of the lumbar puncture and lower extremity electromyography
were within physiological limits. No brain masses or abnormalities were evident at
both magnetic resonance imaging (MRI) and computed tomography (CT) scans. No neural
auto-antibodies (anti-Purkinje cells, anti-granule cells, anti-nucleolin, anti-GABAergic
synapses, DOT-BLOT IgG – Ravo) were detected, neither in serum nor in cerebro-spinal
fluid (CSF). CSF analysis revealed an albumin level of 28,34 mg/dL and an IgG level
of 4,50 mg/dL. The cytology of CSF was negative for tumor cells. In May 2011, an abdominal
CT scan revealed a large pancreatic mass with multiple liver metastases (Fig. 1). Subsequently, a percutaneous liver biopsy revealed pathological features of well-differentiated
neuroendocrine tumor (WDNET) of the pancreas, with a 5 % proliferation index (Ki67).
Serum Chromogranin-A was elevated (524 U/l, upper normal limit 18) and the 111In-octreotide
scintigram resulted positive. An early treatment with monthly intramuscular octreotide
LAR (long-acting releasing) at the dose of 30 mg and daily oral prednisone at the
dose of 25 mg were started. A complete disease restaging was performed in September
2011. As expected, the size of the primary lesion and of liver metastases did not
change significantly, whereas a good biochemical response was detected, with Chromogranin-A
serum level decreasing to 58 U/ml. Clinically, the patient experienced an improvement
in neurological symptoms. However, three months later, neurological symptoms rapidly
worsened, requiring hospitalization. Electroencephalogram (EEG) showed a typical diffuse
encephalopathy pattern, whereas the brain MRI scan was negative. Despite an increase
in the dexamethazone daily dose up to 16 mg, during the hospitalization the cerebellar
syndrome further deteriorated, preventing patient’s self-care. In October 2011, the
ICARS score was 41 and Rankin scale 4 5]. In November 2011, seven months after the onset of the symptoms, patient died from
neurological impairment.

Fig. 1. Contrast computed tomography (CT) images of a patient with paraneoplastic cerebellar
degeneration due to metastatic pancreatic neuroendocrine tumour