Paraneoplastic cerebellar degeneration secondary to ovarian carcinosarcoma: a cerebellar conundrum

Abstract

We present a case of an elderly female patient who presented with a 6-month history of progressive slurred speech, vertigo, unsteadiness and falls. She underwent an extensive battery of neurological and cardiovascular investigations, none of which demonstrated a diagnostic cause for her symptoms. She was referred to the stroke and neurology teams and was started on treatment for presumed anxiety. As her symptoms continued to progress, she was referred to the falls service. Following a multidisciplinary team discussion, she was reviewed by the consultant geriatrician who felt this may be due to a malignancy so the consultant geriatrician arranged blood testsand CT scan of her chest, abdomen and pelvis. These demonstrated a large left adnexal mass and a raised Ca-125 level. The patient was diagnosed with an ovarian tumour, which was treated surgically. A provisional diagnosis of paraneoplastic cerebellar degeneration, secondary to ovarian carcinosarcoma, was made.

An Etiological Investigation of Paraneoplastic Cerebellar Degeneration in Ovarian Cancer Patients: A Systematic Review

“…Among the 102 cases analyzed, a diagnostic process was identified in 20 [4,7,[14][15][16][17][18][19][20][21][22][23][24][25]. Diagnostic measures observed in these studies were grouped into neurological assessments, imaging modalities, laboratory findings, and explorative surgeries performed.…”

“…These cases were followed up with several imaging methods, such as MRI (95%), CT (75%), or ultrasound (40%) to detect cancerous growth or indications of cerebellar dysfunction. All 20 cases where a diagnostic process was identified reported the use of multiple imaging modalities in the diagnostic workup, the most frequent pairing being an MRI with an abdominal CT. A serum or cerebral spinal fluid (CSF) paraneoplastic panel was obtained in 18 out of 20 patients [4,7,[15][16][17][18][19][20][21][22][23]25]. Serum tumor markers, notably cancer antigen (CA) 125, were tested in half of these patients to confirm the presence of a tumor, track tumor growth, or establish remission status [4,7,[15][16][17][18][19][22][23][24].…”

“…Fourteen studies described the long-term effects among OC patients with PCD (n=20) [4,7,[14][15][16][17][18][19][20][21][22][23][24][25]. Among these studies, there were four subgroups: patients who recovered from neuropathy (45%), patients with impairment halted at tumor resolution (15%), patients with progressive neurologic impairment (15%), and patients who succumbed to their condition (15%) [4,7,[14][15][16][17][18][19][20][21][22][23][24][25]. Another key prognostic factor was the presence of anti-Yo, which was evaluated as an indicator of treatment efficacy [22].…”

“…All 20 cases where a diagnostic process was identified reported the use of multiple imaging modalities in the diagnostic workup, the most frequent pairing being an MRI with an abdominal CT. A serum or cerebral spinal fluid (CSF) paraneoplastic panel was obtained in 18 out of 20 patients [ 4 , 7 , 15 - 23 , 25 ]. Serum tumor markers, notably cancer antigen (CA) 125, were tested in half of these patients to confirm the presence of a tumor, track tumor growth, or establish remission status [ 4 , 7 , 15 - 19 , 22 - 24 ].…”

“…The clinical presentation of PCD includes altered gait, diplopia, and difficulty with fine motor skills, with eventual progression to limb and truncal ataxia [ 4 , 5 ]. These symptoms usually occur over several weeks but can progress rapidly in certain cases [ 6 ].…”