Chiari I malformation mimicking myasthenia gravis

Rodolico, Carmelo

doi:10.1136/jnnp.74.3.393

Cited by 10 publications

(3 citation statements)

References 5 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…4 Our patient presented with early prominent ocular symptoms in addition to other symptoms suggestive of a neuromuscular junction disorder, including fatigable proximal limb weakness and slurred speech. 4 Our patient presented with early prominent ocular symptoms in addition to other symptoms suggestive of a neuromuscular junction disorder, including fatigable proximal limb weakness and slurred speech.…”

Section: Discussionmentioning

confidence: 72%

“…3 It commonly presents as headache (80%), occipital or upper cervical pain worsened by valsalva maneuvers (60% to 70%), motor deficits (40% to 74%), sensory changes (50%), clumsiness (15%), dysphagia (10%), and dysphonia (8%). 4 Although various ocular disturbances including oscillopsia, photophobia, eye pain, blurry vision, floaters, transient visual obscurations, nystagmus, diplopia, extraocular muscle weakness, smooth pursuit impairment, blepharoclonus, and visual field deficits are described with Chiari type I malformation, prominent eyelid ptosis as one of the presenting signs of Chiari type I malformation has not been reported in the literature. 4 Although various ocular disturbances including oscillopsia, photophobia, eye pain, blurry vision, floaters, transient visual obscurations, nystagmus, diplopia, extraocular muscle weakness, smooth pursuit impairment, blepharoclonus, and visual field deficits are described with Chiari type I malformation, prominent eyelid ptosis as one of the presenting signs of Chiari type I malformation has not been reported in the literature.…”

mentioning

confidence: 99%

See 1 more Smart Citation

Chiari Type I Malformation

et al. 2011

View full text Add to dashboard Cite

Chiari type I malformation is an unusual differential for sero-negative myasthenia gravis. Magnetic resonance imaging of the brain, carried out in patients with all classical signs and symptoms of myasthenia gravis, helps identify this anomaly. Headaches, although a classic feature of Chiari type I malformation, need not be an early manifestation. Eyelid ptosis as a manifestation of Chiari malformation has not been reported in the literature.

show abstract

Section: Discussionmentioning

confidence: 72%

mentioning

confidence: 99%

Chiari Type I Malformation

et al. 2011

View full text Add to dashboard Cite

show abstract

“…Several other structured lesions of the central nervous system, such as cerebrovascular disease, brain stem tumors and Chiari malformation were described as mimicking MG, due to involvement or compression of the brain stem, could easily lead to misdiagnosis and an inappropriate treatment, but the DWS have not been reported to date 1,2,[6][7][8][9][10][11][12] .…”

mentioning

confidence: 99%

Dandy-Walker syndrome in adult mimicking myasthenia gravis

Cardoso

Lange

Lorenzoni

et al. 2007

Arq. Neuro-Psiquiatr.

View full text Add to dashboard Cite

-The Dandy-Walker syndrome (DWS) is a rare posterior fossa malformation. The DWS can occur associated with other brain or systemic malformations, but ocular abnormalities in this disease are rare and clinical findings mimicking myasthenia gravis have not been described to date. We report a 23-yearold woman who presented mild limitation of the ocular movements with progressive palpebral ptosis, which changed in intensity during the day. The investigation showed negative anti-acetylcholine receptor antibody, repetitive nerve stimulation and "Tensilon test", but the brain magnetic resonance image reveals DWS with hydrocephalus associated with calosal dysgenesis. The characteristic of disease, clinical manifestations and pathologic features, specially the clinical evaluation of ocular abnormalities in suspicion of DWS, including the MG in differential diagnosis are discussed.KEY WORDS: Dandy-Walker syndrome, hydrocephalus, myasthenia gravis, palpebral ptosis. Síndrome de Dandy-Walker em adulto simulando miastenia gravisRESUMO -A síndrome de Dandy-Walker (DWS) é uma rara malformação da fossa posterior que pode ocorrer associada com outras malformações cerebrais ou sistêmicas. As alterações oculares são raras e as manifestações clínicas, simulando miastenia gravis (MG), não foram descritas até o momento. Descrevemos uma mulher de 23 anos apresentando discreta limitação da movimentação ocular com progressiva ptose palpebral que mudava de intensidade durante o dia. A investigação mostrou negativos o anticorpo anti-receptor de acetilcolina, a estimulação nervosa repetitiva e o "teste do Tensilon", porém a ressonância magnéti-ca de crânio revelou DWS com hidrocefalia associada à disgenesia de corpo caloso. As características da doença, manifestações clínicas e patológicas, especialmente a avaliação clínica de anormalidade ocular na suspeita de DWS serão discutidas, incluindo a MG no diagnóstico diferencial.

show abstract