Miller Fisher syndrome--an uncommon clinical presentation.

Miller Fisher syndrome is an uncommon disease and it is a variant of Guillain-Barre syndrome. Miller Fisher syndrome also has rarer variants. Combined features of classic Guillain-Barre syndrome and Miller Fisher syndrome are uncommon. Here we are reporting a case of Miller Fisher variant with Guillain-Barre syndrome overlap in which ataxia, are flexia, oculomotor disturbance and limb weakness occurred within few days.

Hepatocellular Carcinoma with Metastasis to the Cavernous Sinus of Skull Base Causing Ptosis / 대한소화기학회지

The cavernous sinus of skull base is a extremely rare metastastatic site for hepatocellular carcinoma (HCC). A 51-year-old man was diagnosed with HCC by liver biopsy and palliative radiotherapy on HCC including main portal vein was performed. One month later, he was admitted due to sudden onset ptosis. Neurologic findings were normal except for abnormal movement of right eye, and it raised the possibility of abnormality in the right occulomotor, trochlear and the abducens nerves. Contrast-enhanced CT scan of brain showed a mass with homogeneous enhancement involving the right cavernous sinus. T2-weighted axial MR images demonstrated a homogeneous mass with intermediate signal intensity, and contrast-enhanced axial T1-weighted MR images demonstrated a mass with homogeneous enhancement in the right cavernous sinus. We describe a case of HCC metastasis to the cavernous sinus with symptoms of ptosis and disturbance of right eyeball movement.

Hallazgos en la RNM en pacientes con fibrosis congénita generalizada de los músculos extraoculares (CFEOM) / Findings on MRI in patients with congenital generalized fibrosis of the extraocular muscles (CFEOM)

Objetivo: Mostrar los hallazgos en la RNM de pacientes afectados de CFEOM Pacientes y métodos: Se presentan las RNM de 4 pacientes afectados de CFEOM Resultados: En todos se observa una marcada atrofia del recto superior y los otros músculos extraoculares inervados por el III nervio se ven adelgazados. El recto lateral en 1 paciente aparece bilobulado. En un paciente con oftalmoplegia, en la T1 se observa una señal brillante en el interior del cuerpo muscular. Conclusiones: La RNM de estos pacientes con CFEOM muestra 2 características: atrofia muscular del recto superior, elevador del párpado superior y señal brillante en el interior del cuerpo muscular.

Objectives: To show the findings on MRI in patients affected by CFEOM Patients and methods: The MRI of four patients affected by CFEOM were revieved Results: In all cases a significant atrophy of the superior rectus can be seen, and the other extraocular muscles, inervated by the III nerve, are visibly thinner. The lateral rectus, in one patient, appears bilobulated. In another patient with ophthalmoplegy, in T1, a brilliant signal in the interior of the muscular body can be observed. Conclusions: Two characteristics were found on the MRI in these patients with CFEOM. One is the muscular thinness of the PSL and SR, and the other is the brilliant signal in the interior of the muscular body. Also, some structural anomalies can be observed in other extraocular muscles.

Síndrome do seio cavernoso: estudo de 70 casos / Cavernous sinus syndrome: study of 70 cases

Objetivo: Caracterizar as manifestaçöes clínicas e determinar a distribuiçäo epidemiológica e etiológica da síndrome do seio cavernoso (SSC) pela primeira vez no Brasil. Métodos: Os casos de SSC vistos num período de 15 anos foram analizados. Os pacientes foram submetidos a completo exame neurológico e oftalmológico, a extensa propedêutica laboratorial e a exames de imagem do encefálo para determinaçäo da etiologia. Em alguns pacientes foi realizada biópsia cerebral. Resultados: Setenta casos foram encontrados com idades entre 4 e 74 anos (mediana 41). Ambos sexos foram igualmente afetados. Cefaléia ocoreu em 80 por cento dos pacientes, envolvimento do III nervo em 76 por cento, do IV em 43 por cento, do VI em 59 por cento, e do nervo óptico em 46 por cento dos casos. A síndrome de Tolosa-Hunt foi encontrada em 18 pacientes, a paquimeningite hipertrófica em 17, os tumores em 12 , os aneurismas em 11, as fístulas carótido-cavernosas em 10, tuberculoma em um, e tromboflebite em uma paciente. Conclusöes: A SSC ocorre em ambos os sexos e em todos os grupos etários, se manifestando por graves déficits neuro-oftalmológicos. Neuropatia óptica é comum. Inflamaçäo foi a causa mais freqüentemente encontrada da SSC em série.

Síndrome de Miller Fisher: presentación de un caso clínico / Miller Fisher syndrome: review of a clinic case

Presentamos el caso una paciente con síndrome de Miller Fisher, una entidad neurológica infrecuente considerada como una variante del síndrome de Guillain-Barre. El pronóstico aún sin terapéutica parece ser favorable. Se realiza una revisión bibliográfica haciendo particular énfasis en la ubicación de la lesión, métodos paraclínicos y tratamiento actual

Solitary intracranial plasmacytoma of the skull base.

Two cases of extradural solitary plasmacytoma (SIP) with infiltration of the dura, destruction of the skull base, multiple cranial nerve palsies and proptosis are presented. The cases were treated aggressively with surgery and radiotherapy and showed no signs of progressing to multiple myeloma after 15 months and four and a half years respectively.

Electron microscopic study on overacting inferior oblique muscles

Overaction of the inferior oblique(IO) muscle is manifested by elevation of the adducted eye and from the clinical point of view there are two types of overaction. The primary type is of unknown cause, whereas the secondary type is usually related to the palsy of the ipsilateral superior oblique or contralateral superior rectus. An ultrastructural study on the overacting IO muscles was performed compared to normal IO muscles by electron microscopy. Of 16 biopsies of overacting IO muscles, four had primary overacting inferior obliques and twelve had secondary overacting inferior obliques due to paralysis of superior oblique muscle. Additional four IO muscle, obtained from patients with intraocular diseases served as control specimens. The most striking abnormalities were aggregations of mitochondria and degenerating mitochondrial profiles and increased vacuolization in primary and secondary overacting muscles. Many muscle fibers were in different stages of atrophy, and hypertrophy and regeneration of muscle fibers were sometimes visible. The results suggest that the primary overacting IO muscle might be the result of a paresis of the superior oblique muscle.

Alteraçöes da motricidade extrínseca e pupilares nas neuropatias diabéticas / Ocular alterations in diabetic neuropathies

Säo feitas consideraçöes sobre os aspectos clínicos, patologia e prognóstico das alteraçöes da motricidade extrínseca e pupilares nos pacientes diabéticos

Wall-eyed bilateral internuclear ophthalmoplegia (Webino syndrome) and myelopathy in pyoderma gangrenosum

O termo Webino é formado pelas iniciais dos componentes da síndrome (walleyed bilateral internuclear ophthalmoplegia), havendo também perda da convergência ocular. Relatamos o caso de uma paciente de 35 anos de idade, com pioderma gangrenosum, que desenvolveu subitamente mielopatia com nível sensitivo em L1 e, três meses depois, quadro súbito de oftalmoplegia internuclear bilateral, exotropia e perda de convergência. Há poucos casos de síndrome de Webino relatados na literatura, a maioria deles secundária a esclerose múltipla e a doença vascular cerebral. A localizaçäo da lesäo responsável pela síndrome ainda näo está bem estabelecida mas, acredita-se que os fascículos longitudinais mediais e os subnúcleos dos retos mediais do complexo oculomotor no mesencéfalo sejam afetados, embora näo haja ainda confirmaçäo anátomo-patológica. O pioderma gangrenosum é condiçäo caracterizada por úlceras de crescimento rápido e de bordas elevadas com halo eritematoso localizadas preferencialmente na cabeça, tronco e membros inferiores. Embora sua natureza ainda seja desconhecida, parece ser secundário a vasculite subjacente, estando associado a grande variedade de doenças sistêmicas. No caso presente, a ocorrência da síndrome de Webino e de mielopatia sugere que as lesöes no sistema nervoso sejam de natureza vascular, provavelmente secundárias a vasculite. Este é o primeiro relato na literatura de complicaçöes neurológicas do pioderma gangrenosum e da associaçäo de mielopatia à síndrome de Webino