Magnetic resonance imaging in cases 1 and 2 was unremarkable, whereas magnetic resonance angiography demonstrated pathophysiologically significant vertebral basilar disease. The one and a half syndrome is a rare cause of ophthalmoparesis characterized by a conjugate horizontal gaze palsy in one direction and an internuclear ophthalmoplegia in the other. When this occurs, a oneandahalf syndrome plus a 7th nerve palsy, it is termed an eightandahalf syndrome. The eyes display a left exotropia in primary position. The index to diseases and injuries is an alphabetical listing of medical terms, with each term mapped to one or more icd10 codes. This lesion may give rise to a one anda half syndrome with associated ipsilateral facial muscle weakness, also known as eightanda half syndrome. This syndrome is caused by a lesion affecting the pprf and mlf simultaneously, resulting in ipsilateral conjugate gaze palsy and inoref12 a patient with this syndrome. For patients with unilateral or bilateral ino other causes such as traumatic and infectious were found, with neurocysticercosis and brainstem encephalitis being. It presents as a combination of conjugate horizontal gaze palsy in one eye one and internuclear ophthalmoplegia ino of the contralateral eye half. The oneandahalf syndrome is a clinical disorder of extraocular movements characterized by a conjugate horizontal gaze palsy in one direction plus an internuclear ophthalmoplegia in the other. Adie syndrome, also known as holmesadie syndrome, is a neurological disorder characterized by a tonically dilated pupil that reacts slowly to light but shows a more definite response to accommodation i. Before long, eggenberger discovered the combination of one and a half syndrome and ipsilateral facial nerve palsy, and called it as eightanda half syndrome.
Coexisting vertical and horizontal one and a half syndromes. The one and a half syndrome is a rare weakness in eye movement affecting both eyes, in which one cannot move laterally at all, and the other can move in only one lateral direction inward or outward. Atrial myxoma as a cause of one and a half syndrome is very rare and to our limited knowledge this is the first of its type presenting in this rare clinical entity. One and a half syndrome is a disorder of horizontal ocular movement characterized by a lateral gaze palsy on looking toward the side of the lesion and ino on looking in the other direction. Pdf oneandahalf syndrome is characterized by combination of the. Eight and a half syndrome is a rare pontine neuroophthalmologic syndrome made up of a conjugate horizontal gaze palsy, ipsilateral internuclear ophthalmoplegia and ipsilateral lower motor neuron. The main cause of this condition, bilateral renal agenesis, occurs in approximately 1 in 5,000 fetuses and accounts for about 20% of potter syndrome cases. Hypoplastic left heart syndrome is one type of congenital heart defect.
Our case is interesting as this syndrome is one of the most localizing brainstem syndromes. Discussion eight and a half syndrome ehs 1 is the rare association of one and a half syndrome 2 i. One and a half syndrome wikipedia republished wiki 2. The treatment depends on the causes of the syndrome. A seldom reported causal association of two rare entities, an isolated brainstem tuberculoma and an isolated one and a half syndrome in a 12 year old girl is presented. The most common manifestation of this unusual syndrome. The one and a half syndrome is a rare weakness in eye movement affecting both eyes, in which. Objectiveto provide clinicoanatomical correlation for a small pontine tegmental ischemic stroke producing the one and onehalf syndrome associated with. One an dahalf synd rome is a disorder of horizontal ocular movement characterized by a lateral gaze palsy on looking toward the side of the lesion and ino on looking in the other direction. Sheldonhall syndrome genetic and rare diseases information. This pattern is called a one and a half syndrome, one of the manifestations of paramedian pontine dysfunction.
One and a half syndrome lesion on right instant neuro. People with this condition may also have distinctive facial features, extra folds of skin on the neck, and short stature. One anda half syndrome refers to horizontal gaze palsy one with internuclear ophthalmoplegia half due to a lesion of the paramedian pontine reticular formation and medial longitudinal fasciculus. A patient experiencing mucormycosis of the cavernous sinus suffered a sixthnerve palsy and simultaneous carotid artery occlusion with infarction and a contralateral horizontal gaze palsy. Here, we present two patients who presented with clinical features suggestive of eightanda half syndrome along with hemiataxia. A 36yearold woman presented with left gaze limitation and right eye limitation of adduction with horizontal nystagmus on abduction one and a half syndrome in addition to a leftsided lower motor neuron facial nerve palsy figure 1 and video. Frcp harvard medical school overview of tdp43 proteinopathy. Since neither of these structures was within the area of the cysts, it is puzzling as to why they would have been affected by. More formally, it is characterized by a conjugate horizontal gaze palsy in one direction and. A clinical diagnosis of one and a half syndrome secondary to a cerebrovascular event was made. One and onehalf syndrome with supranuclear facial weakness. Illustration of lesions in one and a half syndrome. Eight and a half syndrome by dams ophthalmology faculty duration.
Mri showed an isolated inflammatory granuloma in the brainstem which on empirical treatment with anti tubercular drugs resulted in complete restoration of ocular motility, along with resolution of the lesion on follow up mri at. Shared mechanisms underlying frontotemporal lobar degeneration and amyotrophic lateral sclerosis the f. Botulinum toxin treatment of one and a half syndrome pdf. One and a half syndrome is a syndrome of horizontal movement disorder of both eyes. A patient with bilateral infarction in the mesodiencephalic region showed impairment of all downward rapid eye movements including vestibuloocular movements and foveal smooth pursuit nondissociated downgaze paralysis associated with monocular paralysis of elevation vertical oneandahalf syndrome. A 12yearold girl with eczema not responding to treatment. His concurrent exotropia is a well described, even if uncommon feature of this syndrome and is explained by a tendency of the contralateral eye to drift in the opposite direction due to the horizontal gaze palsy. Intelligence and life expectancy are not usually affected. Oneandahalf syndrome is a gaze abnormality characterized by a conjugate horizontal gaze palsy in one direction plus an internuclear ophthalmoplegia in the other. Eightanda half syndrome is a rare pontine neuroophthalmologic syndrome made up of a conjugate horizontal gaze palsy, ipsilateral internuclear ophthalmoplegia and ipsilateral lower motor neuron.
One anda half plus syndrome is a less known clinical syndrome which constitutes of a conjugate horizontal gaze palsy in one direction and an internuclear ophthalmoplegia in the other direction. Eightandahalf syndrome american academy of ophthalmology. This case talks about one anda half plus syndrome, a clinical syndrome affecting binocular vision and facial nerve. Hypoplastic left heart syndrome hlhs is a birth defect that affects normal blood flow through the heart. Bennett lecture american neurologic association 2008. The one and a half syndrome is characterised by a lateral gaze palsy in one direction and internuclear ophthalmoplegia in the other. Living life to the fullest with ehlersdanlos syndrome pdf. Diplopia 12 blurred vision 8 oscillopsia 4 difficulty looking to one side 2 quivering of the eye 1 no visual complaint 3 table 2. Isolated one and a half syndrome with brainstem tuberculoma. One and a half syndrome an overview sciencedirect topics. This 15yearold patient had a brainstem glioma that caused a gaze palsy to the left right photograph and a left internuclear ophthalmoplegia evident here as incomplete adduction of the left eye on gaze to the right.
Living life to the fullest with ehlersdanlos syndrome pdf free download. In one and a half syndrome, the apparent adduction deficits can be overcome with convergence. Pdf on sep 6, 2011, martin w kurz and others published one anda half syndrome find, read and cite all the research you need on researchgate. Three cases of isolated one and a half syndrome with facial nerve palsy related to infarction are presented. One an dahalf synd rome ohs is characterized by a combination of unilateral horizontal gaze paralysis together with internuclear ophthalmoplegia ino and has three types. Vertical one anda half syndrome with contralesional pseudoabducens palsy in a patient with thalamomesencephalic stroke. The one and a half syndrome is a rare weakness in eye movement affecting both eyes, in which one cannot move laterally at all, and the other can move only in outward direction.
Our patient had a combination of right ino and right facial nerve palsy. A case report of the rare fifteenandahalf syndrome. We describe a patient with a vertical gaze disorder that is in many ways similar to the vertical oneandahalf syndrome except for an upward gaze that affected only the side contralateral to the stroke. One anda half syndrome is characterized by combination of the clinical features of unilateral horizontal gaze palsy and internuclear ophthalmoplegia. A concomitant cn viii palsy in this setting has been called the eightandahalf syndrome. To our knowledge this is the first report of a case of fisher one and a half syndrome with facial paralysis correlated with computed. The mlf connects the abducens nerve on one side with the occulomotor nerve on the contralateral side. Damsdelhi pg medical entrance coaching 9,696 views. Oneandahalf syndrome with its spectrum disorders ncbi. Nuclear magnetic resonance nmr imaging has been used to visualise a localised area of demyelination in the dorsal pons of a patient who presented with an unusual disorder of eye movements, namely, the oneandahalf syndrome. Botulinum toxin treatment of one and a half syndrome. The article is mainly to summarize and analyse the clinical features, pathological anatomy. Oneandahalf syndrome american academy of ophthalmology. Hence we emphasise that myxomas should be always kept in mind in such rare causes of stroke with an.
The patient with horizontal gaze palsy typically is unable to move either eye beyond the. Potter syndrome is a rare disorder, and the exact incidence or prevalence is unknown. May 12, 2014 the one and a half syndrome is a rare ophthalmoparetic syndrome characterized by a conjugate horizontal gaze palsy in one direction and an internuclear ophthalmoplegia in the other. A one an d a half synd rome involves lesions of ipsilateral pprf, or abducens, along with ipsilateral mlf. This syndrome is typically related with damage to the paramedian pontine reticular formation pprf and the medial longitudinal fasciculus mlf on the same side.
Three cases of isolated one anda half syndrome with facial nerve palsy related to infarction are presented. One anda half syndrome referred firstly by fisher in 1967, may result from lesions in the abducens nucleus, in the abducent nerve and mlf, or pprf. Reported boston total cases series brainstem infarct 12 4 16 multiple sclerosis 2 14 16 pontine glioma 2 1 3 arteriovenous malformation 1 0 1 pontine hemorrhage 8 0 8 basilar artery aneurysm 0 1 1 cerebellar astrocytoma 2 0 2 metastatic melanoma 1 0 1 ependymoma fourth ventricle 1 0 1 29 20 49 table 1. Physical examination showed right one anda half syndrome figure 1ai and video 1, lower motor neuron weakness of the right face muscles video 2, hemifacial spasm on the left side of the face video 3, bilateral hearing loss and. Causes for the one and a half syndrome were most frequently vascular infarct and hemorrhage, demyelinating and neoplastic. One an dahalf synd rome is a gaze abnormality characterized by a conjugate horizontal gaze palsy in one direction plus an internuclear ophthalmoplegia in the other. Fischer syndrome neurology a unilateral pontine lesion involving the medial longitudinal fasciculus and the pontine paramedian reticular formation, resulting in ipsilateral gaze palsy and internuclear ophthalmoplegia on contralateral gaze.
A full ocular motility exam, including convergence, should be performed. Causes of internuclear ophthalmoplegia with a conjugate gaze paralysis have included infarcts, 1 4 gliomas of the brainstem, 3,4 vascular malformations, 4 and demyelinating disease. Article pdf available in ideggyogyaszati szemle 601112. One anda half syndrome is a clinical disorder characterized by an ipsilateral conjugate horizontal gaze palsy the one and an ipsilateral internuclear ophthalmoplegia ino the half. As the baby develops during pregnancy, the left side of the heart does not form correctly. The one and a half syndrome is a clinical disorder of extraocular movements characterized by a conjugate horizontal gaze palsy in one direction plus an internuclear ophthalmoplegia in the other. Pdf the oneandahalf syndromea unilateral disorder of the. Vertical halfandahalf syndrome journal of neurology.
In left gaze, the right eye does not cross the midline and the left eye has a normal excursion. Vincent abstract the authors describe a case of a tenyearold boy with one an dahalf synd rome secondary to a lesion at the dorsal tegmentum in the lower pons, and comment on the horizontal gaze physiology. Oneandahalf syndrome is characterized by conjugate horizontal gaze palsy in. There is also interruption of internuclear fibres of. Jan 29, 2008 one and a half syndrome is a gaze abnormality characterized by a conjugate horizontal gaze palsy in one direction plus an internuclear ophthalmoplegia in the other. It is due to a unilateral lesion of the dorsal pontine. The presence of a concomitant oaah syndrome along with a facial nerve palsy has been called the eight. Vertical and horizontal one and a half syndrome with.
The one andahalf syndrome9 oahs is an ocular movement disorder characterized by lateral gaze palsy to one direction associated with internuclear. One and a half syndrome is a clinical disorder characterized by an ipsilateral conjugate horizontal gaze palsy the one and an ipsilateral internuclear ophthalmoplegia ino the half. The common symptoms are double vision and oscillopsia. The case described here fits the criteria needed for a previously named variant of the eaah syndrome, the sixteen. One anda half syndrome is characterized by conjugate horizontal gaze palsy in one direction in addition to contraversive internuclear ophthalmoplegia. I have been following this protocol for over a year and a half and at the age of 65, the.
To describe a second type of the one an dahalf synd rome in which adduction rather than abduction of one eye is the preserved horizontal movement. Mar 08, 2018 this lesion may give rise to a one and a half syndrome with associated ipsilateral facial muscle weakness, also known as eight and a half syndrome. The pprf stimulates the abducens nerve ipsilaterally. More formally, it is characterized by a conjugate horizontal gaze palsy in one direction and an internuclear ophthalmoplegi. A guide to an intimate relationship with a partner who has asperger syndrome maxine c. The syndrome is usually due to a single unilateral lesion of the paramedian pontine reticular formation or the abducens nucleus on one side. Potter syndrome nord national organization for rare disorders. A concomitant cn viii palsy in this setting has been called the eightanda half syndrome. We report a case who presented with this constellation of findings with diffusion weighted images dwi and apparent diffusion coefficient adc changes corresponding to the anatomical correlate in the dorsal pontine tegmentum.
Nuclear magnetic resonance nmr imaging has been used to visualise a localised area of demyelination in the dorsal pons of a patient who presented with an unusual disorder of eye movements, namely, the one and a half syndrome. One and a half syndrome wikimili, the free encyclopedia. The most common manifestation of this unusual syndrome is limitation of horizontal eye movement to abduction of one eye with no horizontal movement of the other eye. Oct 06, 2014 video shows patient with one and half syndrome. The one an d a half synd rome is a rare weakness in eye movement affecting both eyes, in which one cannot move laterally at all, and the other can move in only one lateral direction inward or outward. Nuclear magnetic resonance nmr imaging has been used to visualise a localised area of demyelination in the dorsal pons of a patient who presented with an unusual disorder of eye movements, namely, the one anda half syndrome. An unusual presentation of left atrial myxoma as one and a.
Motility exam showed a left gaze palsy and left eye limitation of adduction. The only remaining horizontal movement is contralateral abduction. Supranuclear ocular movements comprise chiefly vertical and horizontal movements. One and a half syndrome complicating resection of fourth. A 50yearold man who enjoyed good health in the past was admitted for diplopia, acute hearing loss of both ears and leftsided limb clumsiness on waking up. When this lesion also affects the fascicle of the the ipsilateral facial nerve cn vii in the region of the facial colliculus as it wraps around the cn vi nucleus, it produces a lower motor neuron pattern of ipsilateral facial weakness. This syndrome consists of the oneandahalf syndrome plus lower motor neuron pattern facial weakness on the side ipsilateral to the ino. Classic horizontal one and a half syndrome is commonly caused by a vascular accident occurring in the lower pons involving the paramedian pontine reticular formation and the medial longitudinal fasciculus. One anda half syndrome is a rare disorder in which a lesion affects the horizontal gaze center and the medial longitudinal fasciculus on the same side. Pdf vertical oneandahalf syndrome with contralesional. Horizontal gaze palsy in oneandahalf syndrome may result from lesions in the abducens nucleus, in the abducens nerve and the medial longitudinal fasciculus. We named these findings the vertical halfandahalf syndrome. The one an dahalf synd rome is a clinical disorder of extraocular movements characterized by a conjugate horizontal gaze palsy in one direction plus an internuclear ophthalmoplegia in the other. Apr 23, 2018 one and a half syndrome is characterized by conjugate horizontal gaze palsy in one direction in addition to contraversive internuclear ophthalmoplegia.
More formally, it is characterized by a conjugate horizontal gaze palsy in one direction and an internuclear ophthalmoplegia in the other. Total clinical recovery occurred at the end of 6 months. Jan 20, 2016 sheldonhall syndrome, also known as distal arthrogryposis type 2b, is characterized by joint deformities contractures that restrict movement in the hands and feet. Jun 16, 2016 the one and a half syndrome is a clinical disorder of extraocular movements characterized by a conjugate horizontal gaze palsy in one direction plus an internuclear ophthalmoplegia ino in the other direction fisher 1967. One anda half syndrome is a syndrome of horizontal movement disorder of both eyes. Vincent abstract the authors describe a case of a tenyearold boy with oneandahalf syndrome secondary to a lesion at the dorsal tegmentum in the lower pons, and comment on the horizontal gaze physiology. The exact anatomical and physiological mechanism underlying vertical gaze still remains obscure. What are the signs and symptoms of oneandahalf syndrome. Before long, eggenberger discovered the combination of one and a half syndrome and ipsilateral facial nerve palsy, and called it as eight and a half syndrome.