This disorder is characterized by distinctive craniofacial malformations that occur in association with limb abnormalities. We present 3 cases studied at the puebla general hospital in one year his treatment and evolution. Recurrences of gbs are well documented but repeated episodes of mfs are very rarely seen in clinical practice. Millerfisher variant of guillain barre syndrome is a rare variant of a rare disease that affects the nervous system of people. In some cases contemporary involvement of the central nervous system has been described cns. This case was in the aers database and is included in the current report. Millerfisher syndrome associated with chronic lymphocytic. The miller fisher syndrome mfs is generally considered to be a disease of the peripheral nervous system.
Click on any term below to browse the alphabetical index. People with lissencephaly have an abnormally smooth brain with fewer folds and grooves. Department of neurology, kansai electric power hospital, osaka city, osaka, japan. It makes up just 1 to 5 percent of guillainbarre cases in the western world. Miller fisher syndrome mfs is a subgroup of a more common yet still rare nerve disorder known as guillainbarre syndrome gbs. Miller fisher december 5, 19, waterloo, ontario april 14, 2012, albany, new york was a canadian neurologist whose notable contributions include the first detailed descriptions of lacunar strokes, the identification of transient ischemic attacks as stroke precursors, the identification of the link between carotid atherosclerosis and stroke, and. It is largely a clinical diagnosis based on the cardinal clinical features of ophthalmoplegia, ataxia and areflexia. Miller fisher syndrome is a rare, acquired nerve disease that is considered to be a variant of guillainbarre syndrome. Guillainbarre and miller fisher syndromes occurring with. A 50yearold man presented with a brief history of slurred speech, unsteadiness, double vision and paraesthesia. What is miller fisher syndromecausessymptomstreatment. Miller fisher syndrome electrodiagnosis slideshare. We report a pa tient with some evidence in support of cerebellar involvement.
Statistics of miller fisher syndrome 4 people with miller fisher syndrome have taken the sf36 survey. Weakness of the eye muscles also known as ophthalmoplegia. Millerdieker syndrome is a condition characterized by a pattern of abnormal brain development known as lissencephaly. Clinical features and prognosis of miller fisher syndrome neurology. Pdf intravenous immunoglobulin therapy for miller fisher. Millerfisher syndrome associated with chronic lymphocytic leukemia zeynep ak. Miller fisher syndrome is a rare variant of guillainbarre syndrome resulting in a postinfectious ne. Symptoms often start several days after a viral illness. We report a case in which it was possible to prove involvement of cranial nerves vii, viii, ix and x and to exclude cns involvement.
The main features are lack of muscle coordination ataxia, eye muscle weakness resulting in the inability to move the eyes in several directions ophthalmoplegia, and the absence of tendon reflexes. Two of them will have the miller fisher syndrome in this century. Millerdieker syndrome mds is a genetic condition characterized by a specific brain malformation lissencephaly. Miller fisher syndrom abstract miller fisher syndrom is a guillainbarre variety characterized for oftalmoplejia, arreflexia and ataxia. This syndrome is defined by the triad of ophthalmoplegia, areflexia, and ataxia 1. Gq1b antibody in serum is an excellent diagnostic marker for mfs. Several mr imaging studies also have shown cns abnormalities in mfs. Because the laboratory findings of the millerfisher syndrome are nonspecific, its the. We analyzed clinical recovery of 92 patients with miller fisher syndrome who had been treated with iv immunoglobulin ivig. Miller fisher syndrome and polyneuritis cranialis in covid. Miller syndrome is a rare condition that mainly affects the development of the face and limbs.
Total score ranges from 0 to 3,600 being 0 the worst and 3,600 the best. The authors report a case of a 27 year old male patient who has exhibited external ophthalmoplegia, anisocoria, ataxia, absent deep reflexes, dysphagia, dysphonia, bilateral peripheral facial paralysis, hypopallesthesia and a mild sensory impairment of the four extremities. Miller fisher syndrome is a rare acquired nerve disease considered to be a variant of guillainbarre syndrome. Pdf miller fisher syndrome is an uncommon disease and it is a variant of guillainbarre syndrome. The annual incidence of miller fisher syndrome is about 0. Pdf miller fisher syndrome an uncommon clinical presentation.
Atypical miller fisher syndrome with anisocoria and. Double vision is usually the first sign of the weakened eye muscles. Millerfisher syndrome can be distinguished from guillain barre syndrome by a few clinical features. Does any member of your family have miller fisher syndrome or may be more predisposed to developing the condition. Pupillary involvement is common in mfs and has been reported in 3542% of mfs patients. A variant of the guillainbarre syndrome characterized by the acute onset of oculomotor dysfunction, ataxia, and loss of deep tendon reflexes with relative sparing of strength in the extremities and trunk. Serological testing was positive for gq1b antibody in keeping with the diagnosis of miller fisher syndrome mfs. Very few affected children survive beyond childhood. Miller fisher syndrome mfs is characterized by the clinical triad of ophthalmoplegia, ataxia, and areflexia, and is considered a variant form of guillainbarre. Bickerstaffs brainstem encephalitis, miller fisher. The severity of this disorder varies among affected individuals.
Miller fisher syndrome also has rarer variants find. On examination, there was ophthalmoplegia, nystagmus, areflexia and lower limb and gait ataxia. It is important to recognize the clinical features of mfs so that appropriate therapy may be instituted. Ivig slightly hastened the amelioration of ophthalmoplegia and ataxia. Miller fisher syndrome mfs is a rare, acquired nerve disease that is considered to be a variant of guillainbarre syndrome. With poliomyelitis under control in developed countries, gbs is now the most important cause of acute flaccid paralysis. It is a form of guillainbarre syndrome gbs and named after a wellknown neurologist, dr. Miller fisher syndrome is considered to be a variant of guillainbarre syndrome.
Pdf millerfisher syndrome is characterized by gait ataxia, external ophtalmoplegia and areflexia and thought as an uncommon variant of guillain barre. Causes miller fisher syndrome and treatment for miller fisher syndrome. It is characterized by abnormal muscle coordination, paralysis of the eye muscles, and absence of the tendon reflexes. We report a 52yearold patient with miller fisher syndrome and discuss wernickes encephalopathy as one important differential diagnosis. Sitajayalakshmi s, borgohain r, mani j, mohandas s department of neurology, nizam institute of medical sciences. Although laboratory studies may lend support, the diagnosis remains a clinical one. Miller fisher syndrome mfs is characterised by a clinical triad of ophthalmoplegia, ataxia and areflexia, and is considered to be a variant of gbs. They often have an opening in the roof of the mouth cleft palate andor a split in the upper lip. Millerfisher syndrome contributed by jason barton, university of british columbia, june 2008. Our two cases highlight the rare occurrence of miller fisher syndrome and polyneuritis cranialis during the covid2 pandemic. Mean of miller fisher syndrome is 1811 points 50 %. Miller fisher syndrome electrodiagnosis dr mohamed ibrahim khalil physical medicine rheumatology alexandria university, egypt to contact dr m.
Although case reports have discussed isolated ophthalmoplegia as a presentation of mfs, anisocoria and rapid fluctuation of pupillary diameter have not been reported. Here you can see if miller fisher syndrome can be hereditary. Miller fisher syndrome mfs is a disease of the nerves. Two weeks later, both patients made a complete neurological recovery, except for residual anosmia and ageusia in the first case. Miller fisher syndrome, a variant of an acute inflammatory neuropathy is often associated with serum antibodies to the ganglioside gq1b, but the pathogenic role of these antibodies and other. The cause is an autoimmune reaction which may be triggered by an infection but more often than not the cause is not found.
Miller fisher miller fisher syndrome mfs is characterized by three conditions. He had been unwell for 12 days with campylobacter gastroenteritis. Millerfisher syndrome mfs is a rare cranial nerve variant of guillainbarre syndrome gbs. Clinical features of millerfisher syndrome in pregnancy.
While gbs affects just 1 person in 100,000, mfs is even more uncommon. Files are available under licenses specified on their description page. Miller fisher syndrome also known as fisher s syndrome, cranial variant of guillainbarre syndrome and cranial variant of gbs is an acquired disease of the nerves and is a rare disease. Manabu inoue department of neurology, kansai electric power hospital, 217 fukushima, fukushimaku, osaka city, osaka, japan. It affects men twice as commonly as women, and occurs at any age, including childhood 2. Miller fisher syndrome is a rare variant of guillainbarre syndrome resulting in a postinfectious neuropathy that affects 0. Similar to guillainbarre syndrome, the symptoms of mfs may be preceded by a viral illness. Bickerstaff brainstem encephalitis is a rare inflammatory disorder of the central nervous system, first described by edwin bickerstaff in 1951. A medical literature search in pubmed yielded 1 case report of guillainbarre. Children with miller syndrome are born with underdeveloped cheek bones malar hypoplasia and a very small lower jaw micrognathia. Miller fisher syndrome mfs is an uncommon variant of guillain barre syndrome gbs. Miller fisher syndrome associated with polymyoclonus. Miller fisher syndrome mfs, a variant of the guillainbarre syndrome gbs, is characterized by ophthalmoplegia, ataxia, and areflexia.
Miller fisher syndrome fisher syndrome information. Mfs is a rare disorder that is characterized by the acute onset of ophthalmoplegia, ataxia, and areflexiahyporeflexia. Millerfisher syndrome mfs is an uncommon variant of guillainbarre syndrome gbs. Miller fisher syndrome is a variant of guillainbarre syndrome characterized by the classic triad of ophthalmoplegia, ataxia, and areflexia. We use cookies to offer you a better experience, personalize content, tailor advertising, provide social media features, and better understand the use of our services. An antigq1b antibody syndrome has been proposed to underlie the common pathophysiology for the three disorders. Mds is caused by a deletion missing piece of genetic material on the short arm of chromosome 17 17p. Miller syndrome is a very rare genetic condition often referred to as postaxial acrofacial dysostosis. Like guillainbarre syndrome, symptoms may be preceded by a viral illness. Normally the exterior of the brain cerebral cortex is multilayered with folds and grooves.
47 340 1220 459 458 896 1144 1273 1043 735 1274 884 605 447 890 778 1418 1448 319 984 874 222 393 827 1296 1151 954 1000 956 244 654 861