Oneandahalf 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. The one and a half syndrome is a rare weakness in eye movement affecting both eyes, in which one cannot move laterally at all, the other can move only in outward direction. Sandifer syndrome a rare disorder that usually affects children up to the ages of 18 to 24 months. This syndrome is typically related with damage to the paramedian pontine reticular formation pprf and the medial longitudinal fasciculus mlf on the same side. To report a case of eightandahalf syndrome presenting with a conjugate horizontal gaze palsy,an ipsilateral internuclear. The cause of noonan syndrome in 15 to 20 percent of people with this disorder is.
Differentiat ion syndrome occurred as early as 1 day and up to 3. Of the 34 patients with relapsed or refractory aml who experienced differentiation syndrome, 27 79% patients recovered after treatment or after dose interruption of tibsovo. Nystagmus is present when the eye on the opposite side of the lesion is abducted. 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.
On the basis of the oneandahalf syndrome, there are a series of related rare syndromes called the. This ms patient had fishers oneandahalf syndrome characterized by. Lippincott journals subscribers, use your username or email along with your password to log in. Eightandahalf syndrome is a rare condition involving the ipsilateral abducens nucleus or paramedian pontine reticular formation pprf. It is usually associated with physical growth delays, mild to moderate intellectual disability, and characteristic facial features. 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. Botulinum toxin treatment of one and a half syndrome. Fishers one and a half syndrome eccles health sciences. Down syndrome ds or dns, also known as trisomy 21, is a genetic disorder caused by the presence of all or part of a third copy of chromosome 21. Sos1 gene mutations cause an additional 10 to 15 percent, and raf1 and rit1 genes each account for about 5 percent of cases. Fisher 115 introduced the term oneandahalf syndrome to refer to a paralysis of eye movements in which one eye lies centrally and fails completely to move horizontally while the other eye lies in an abducted position and cannot be adducted past the midline.
Sjogrens syndrome american college of rheumatology. Three cases of isolated oneandahalf syndrome with facial nerve palsy related to infarction are presented. There have been cases of improvement in extraocular movement with botulinum toxin injection. This syndrome consists of the oneandahalf syndrome plus lower motor neuron pattern facial weakness on the side ipsilateral to the ino. One and a half syndrome an overview sciencedirect topics. Whitenose syndrome wns is a disease that affects hibernating bats. Oneandahalf syndrome american academy of ophthalmology. 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. Since neither of these structures was within the area of the cysts, it is puzzling as to why they would have been affected by. The most common manifestation of this unusual syndrome. Mahale 19 reported two cases of nine syndrome in 2015. The syndrome is usually due to a single unilateral lesion of the paramedian pontine reticular formation pprf or the abducens nucleus on one side causing the. A 58yearold africanamerican woman presented with blurry vision and foreign body sensation in the left eye.
The only remaining horizontal movement is contralateral abduction. Cranial nerve involvement i 0 ii 1 iii 0 v 3 vii 4 viii 2 ix 3 xi 0 xii 2 horners syndrome 1 weakness or spasticity 6 sensory deficits 7 abnormally brisk or asymmetric reflexes 5 extensor plantar responses 9. Magnetic resonance imaging in cases 1 and 2 was unremarkable, whereas magnetic resonance angiography demonstrated pathophysiologically significant vertebral basilar disease. Mutations in multiple genes can cause noonan syndrome. Living life to the fullest with ehlersdanlos syndrome pdf free download. The code is valid for the year 2020 for the submission of hipaacovered transactions. A neurological exam with attention to the cranial nerves may help you further localize a lesion. Pdf the oneandahalf syndromea unilateral disorder of the. It is characterized by dysfunction and destruction of the exocrine glands associated with lymphocytic infiltrates and immunological. 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.
A 52 year old man with hypertension and diabetes mellitus presented with sudden onset of binocular diplopia on looking to the left side, right facial weakness, and epiphora in the right eye. Eightandahalf syndrome american academy of ophthalmology. Final diagnosis of oneand ahalf plus syndrome was made, i. Pdf the oneandahalf syndrome is a clinical disorder of extraocular. Eight and a half syndrome with hemiparesis and hemihypesthesia. The fungus geomyces destructans is known to be associated with wns.
Oneandahalf syndrome is a disorder of horizontal ocular movement characterized by a lateral gaze palsy on. It is usually caused by a single unilateral lesion of the paramedian pontine reticular formation or the abducens nucleus causing the. A left horizontal gaze palsy with gaze evoked nystagmus a left internuclear ophthalmoplegia on gaze right with adduction weakness os abducting nystagmus od full vertical gaze upbeat nystagmus on upgaze ill sustained convergence normal the oneanda. Oneandahalf syndrome with its spectrum disorders ncbi. Pdf the authors describe a case of a tenyearold boy with oneandahalf syndrome secondary to a lesion at the dorsal tegmentum in the lower pons.
The oneandahalf syndrome is characterised by a lateral gaze palsy in one direction and internuclear ophthalmoplegia in the other. The treatment depends on the causes of the syndrome. 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. Case report of a patient with oneandahalf plus syndrome.
Mutations in other genes each account for a small number of cases. 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. More formally, it is characterized by a conjugate horizontal gaze palsy in one direction and an internuclear ophthalmoplegia in the other. Inlyta and none of the patients receiving sorafenib see adverse reactions 6. It is due to a unilateral lesion of the dorsal pontine tegmentum, involving the ipsilateral paramedian pontine reticular formation, internuclear fibres of the ipsilateral medical longitudinal fasciculus and, usually, the abducens nucleus. It felt like a miracle because i had been walking around like that for over a year and none of the many medical professionals. 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. We report a 71yearold man who presented with eight and a half syndrome. The oneandahalf syndromea unilateral disorder of the pontine tegmentum.
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. Facial nucleusfascicles in the lower pontine tegmentum contributed to the observed clinical signs. In the list of named numerical neuroophthalmological syndromes, such as oneandahalf syndrome and others, we report for the first time twentyandahalf syndrome, which is characterized by oneandahalf syndrome with bilateral seventh and right fifth nerve palsy 1. I have been following this protocol for over a year and a half and at the age of 65, the time i have put into it has been totally worth it. Oneandahalf syndrome with facial diplegia, also referred to as the fifteenandahalf syndrome, is an extremely rare clinical entity caused by involvement of bilateral tegmentum of pons. More than one half of patients with the condition will have a missing x chromosome 45,x in all cells studied or a combination of monosomy x and normal cells 45,x46,xx.
Pdf the oneandahalf syndrome is characterised by a lateral gaze palsy in one direction and internuclear. Mutations in the ptpn11 gene cause about half of all cases. Ocular motor examination revealed combination of right gaze paresis and right internuclear ophthalmoplegia suggestive of horizontal oneandahalf syndrome fig 1ac. It causes unusual movements in a childs neck and back that sometimes make it look like they. Oneandahalf syndrome, which is a clinical disorder that is characterized by conjugate horizontal gaze palsy in one direction the one and an internuclear ophthalmoplegia in the other the onehalf, was first described by fisher 2 in 1967. The syndrome was first documented in new york in the winter of 20062007. The immediate treatment with antiaggregating therapy acetylsalicylic acid 300. Herein, we report a 52yearold male who presented with oneandahalf syndrome with left facial paralysis, which was. Their patient had bilateral conjugate upgaze palsy and a right unilateral downward palsy suggestive of vertical one and a half syndrome with left horizontal one and a.
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. Vertical and horizontal one and a half syndrome with. A clinical diagnosis of one and a half syndrome secondary to a cerebrovascular event was made. One and a half syndrome complicating resection of fourth. Forced ductions may be helpful to rule out a restrictive palsy.
Oneandahalf syndrome is a clinical disorder characterized by an ipsilateral conjugate horizontal gaze palsy the one and an ipsilateral internuclear ophthalmoplegia ino the half. This case report is the first to describe a traumatic cause of oneandahalf syndrome due to direct injury to the pontine tegmentum from a penetrating head injury, and illustrates the importance of clinicoradiological findings in patient workup and management. The oneandahalf 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. In clinical trials with inlyta, arterial thromboembolic events including transient ischemic attack, cerebrovascular accident, myocardial infarction, and retinal artery occlusion were reported in. The name describes the manifestation of a white fungus that appears on the muzzle and other body parts of bats. Living life to the fullest with ehlersdanlos syndrome pdf. 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 45yearold asian hindu woman presented with vomiting and. In oneandahalf syndrome, the apparent adduction deficits can be overcome with convergence.
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