Posterior reversible encephalopathy syndrome following immunoglobulin therapy in a patient with Miller-Fisher syndrome

A correct diagnosis of the pedunculated lesion may be difficult , despite the typical radiological presentation, because of the limitation in define the origin of the mass, since a thin pedicle may be almost undetectable at images (1,4,5). The most used modalities of imaging in diagnosis include US, CT and MRI (1–4,6,8). At US, the image is typically hyperechoic, homogeneous, with well defined margins; and, in cases of giant lesions, central heterogeneity may be present (8). At CT, with a certain frequency, giant hemangiomas do not present with the typical pattern of hypoattenuating lesion with centripetal enhancement and homogenization at delayed sections, due to the presence of avascular areas of necrosis, fibrosis or hemorrhage (3,8). MRI is the most sensitive and specific (> 90%) diagnostic method (4,6). The lesions are well defined, homogeneous, with low signal intensity at T1-weighted sequences, and high signal intensity at T2-weighted sequences. Biopsy is not recommended in such cases, due to the risk of hemorrhage (6). There are reports in the literature describing pedunculated hemangiomas as gastric, adrenal tumor (1,4) , retroperitoneal mass (1) , other pedunculated liver tumors such as hepatocellular carcinoma, mesenchymal hamartoma, focal nodular hyperplasia or adenoma (4). Surgical treatment is reserved for cases of giant or symptomatic lesions, uncertain diagnosis, lesions with complications (1,2,4–7) , and for cases of pedunculated hemangiomas due to their tendency to torsion (5,6). REFERENCES 1. Ha CD, Kubomoto SM, Whetstone BM, et al. Pedunculated hepatic he-mangiomas often misdiagnosed despite their typical findings. A case of pedunculated hepatic he-mangioma mimicking submucosal tumor of the stomach. Torsion of a giant pedunculated liver hemangioma mimicking acute appendicitis: a case report. World J Emerg Surg. 2010;5:2. 6. Guenot C, Haller C, Rosso R. Hémangiome caverneux pédiculé géant du foie: à propôs d'un cas et revue de la littérature. A 54-year-old female patient presenting with ophthalmopa-resis, ataxia and areflexia for one week. The patient denied fever, muscle weakness, and did not report any previous comorbidity. At physical examination, she was normotensive, oriented, with bilateral flexor cutaneous-plantar reflex and preserved superficial/deep sensitivity. Human immunodeficiency virus, Epstein-Barr virus, cytomegalovirus, HTLV-1 and VDRL serologies were negative. Considering such findings, the hypothesis of Miller-Fisher syndrome was raised, and liquor cerebrospinalis analysis demonstrated hyperproteinorachia, confirming the diagnosis. Within 24–48 hours after immunoglobulin therapy initiation, the patient presented with intense headache followed by tonic-clonic seizures and later decreased level of consciousness, with no association with hypertensive peaks. …


Dear Editor,
A 54-year-old female patient presenting with ophthalmoparesis, ataxia and areflexia for one week. The patient denied fever, muscle weakness, and did not report any previous comorbidity. At physical examination, she was normotensive, oriented, with bilateral flexor cutaneous-plantar reflex and preserved superficial/deep sensitivity. Human immunodeficiency virus, Epstein-Barr virus, cytomegalovirus, HTLV-1 and VDRL serologies were negative. Considering such findings, the hypothesis of Miller-Fisher syn-drome was raised, and liquor cerebrospinalis analysis demonstrated hyperproteinorachia, confirming the diagnosis.
Within 24-48 hours after immunoglobulin therapy initiation, the patient presented with intense headache followed by tonic-clonic seizures and later decreased level of consciousness, with no association with hypertensive peaks. Magnetic resonance imaging (MRI) ( Figure 1A,B,C) showed sparse hyperintense areas in the white substance, bilaterally on T2-weighted and FLAIR sequences, predominantly in the parieto-occipital regions, without diffusion restriction and without gadolinium enhancement, demonstrating an imaging pattern suggestive of posterior reversible encephalopathy syndrome (PRES). After the therapy suspension and adoption of support measures, the patient progressed satisfactorily, with no sequelae and reversion of the MRI findings ( Figure 1D). The Brazilian radiological literature has recently highlighted the relevant role played by MRI in the improvement of the diagnosis of central nervous system conditions (1)(2)(3)(4)(5) .
MRI findings are quite suggestive and characterized by hyperintense areas on T2-weighted and FLAIR sequences, in general affecting the white substance bilaterally and symmetrically, with predilection for the parieto-occipital region. It may also affect the frontal lobes, internal and external capsules, cerebellum and encephalic trunk (7)(8)(9) . At early stages of the condition, diffusion MRI does not demonstrate any abnormalities, but inappropriate management may result in irreversible damages presented as diffusion restriction corresponding to cytotoxic edema.
Recent studies by means of retrospective analysis, utilizing MRI and laboratory data, have demonstrated the association between PRES and albumin serum levels. There are evidences that significantly decreased albumin serum levels lead to a higher risk to develop vasogenic-type edema (12) . This is due to the fact that, in conditions with endothelial damages caused by inflammatory processes, the decrease in the colloidosmotic pressure, directly related to the albumin levels, may facilitate the development of vasogenic edema. Thus, the early administration of human serum albumin might prevent ischemic damages and reduce possible sequelae (12) .
Finally, despite being rare after administration of immunoglobulin, PRES should be considered in cases where typical MRI findings are present. One should not wait until the onset of a hypertensive episode to take such a diagnostic possibility into consideration.