Case Report

Ataxia, Ophthalmoplegia, and Areflexia: What Would You Think?

Table 1

Cases of proven carcinomatous meningitis presenting as Miller Fisher syndrome.

AuthorTumourPresentationCSFNCSImagingAnti-GQ1bOutcomePostmortem findings if performed

Guarino et al. [50]Stomach adeno-carcinomaSix months after gastrectomy with three days of diplopia, occipital headache. OE: bilateral VI nerve palsies, severe ataxia, and global areflexiaProt 0.6#x2009;g/L,
WCC-1/mL, glu-0.34 g/L.
cytology adenocarcinomatous cells
CT normalTreated with chemotherapy but worsened and died 20 days later
Myeloma4 days of diplopia and diffuse arthralgia. Past history of thyroidectomy for cancer 4 years prior and AML 2 years prior. OE: bilateral III nerve palsies, ataxia, and global areflexiaProt 1 g/L,
WCC-290/mL (lymphs),
glu-“normal”,
Cytology myeloblasts
CT normalTreated with intrathecal chemotherapy but died 40 days later

Nakatsuji et al. [51]Signet ring adeno-carcinoma of unknown primaryOne month progressive diplopia and unsteadiness. OE: bilateral opthalmoplegia, sluggish pupil light responses, hypo/areflexia, ataxia, and decreased sensationOpening pressure 12 cm/H20,
Prot 2.7 g/L
WCC 51 (65% lymphs, 35% polys),
Glu-6.4 g/L,
Cytology negative
Absent sural/ tibial potentials, and normal motor conductionMRI with contrast enhancement of III, IV, VI, XII, ponsRx with IvIg but worsened and died nine weeks after admissionBrain leptomeningeal, cranial nerve, and choroid plexus dissemination of signet right adenocarcinoma

Csépány et al. [52]Bronchial adeno-carcinomaFive days of clumsiness of the right arm, double vision, and unsteadiness. OE: right VI nerve palsy, bilateral VII, decreased reflexes, and ataxiaGlu 3.1 g/L,
WCC 256 (20% lymphs, 48% macros, 12% monos, 20% large atypical cells, and a few signet ring cells
Mild axonal sensory-motor neuropathyCT contrast-right sylvian fissure enhancement. MRI few small enhancing cortical regions.
CT chest, small lung tumour