Vertical oneandahalf syndrome clinical, oculography and. This syndrome is typically related with damage to the paramedian pontine reticular formation pprf and the medial longitudinal fasciculus mlf on the same side. Living life to the fullest with ehlersdanlos syndrome pdf free download. The exact anatomical and physiological mechanism underlying vertical gaze still remains obscure. One and a half syndrome an overview sciencedirect topics. We believe that our patients weakness resulted from involvement of the fourth bundle of dejerine in the pontine tegmentum.
Shared mechanisms underlying frontotemporal lobar degeneration and amyotrophic lateral sclerosis the f. 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. 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. 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.
The patient with horizontal gaze palsy typically is unable to move either eye beyond 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. 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 only in outward direction. Supranuclear downgaze paralysis with monocular elevation palsy, better known as vertical oneandahalf syndrome, results from lesions affecting the mesodiencephalic region. Magnetic resonance imaging in cases 1 and 2 was unremarkable, whereas magnetic resonance angiography demonstrated pathophysiologically significant vertebral basilar disease. Sheldonhall syndrome, also known as distal arthrogryposis type 2b, is characterized by joint deformities contractures that restrict movement in the hands and feet. Diplopia 12 blurred vision 8 oscillopsia 4 difficulty looking to one side 2 quivering of the eye 1 no visual complaint 3 table 2. Bennett lecture american neurologic association 2008. Judged clinically the patient made a nearly complete recovery, but little change was apparent in the nmr image six months later. More formally, it is characterized by a conjugate horizontal gaze palsy in one direction and an internuclear ophthalmoplegia in the other. A 58yearold africanamerican woman presented with blurry vision and foreign body sensation in the left eye. Conceptual medicine by dr prathap bingi 11,059 views. The underlying pathological lesion is frequently at the level of the unilateral tegmentum, affecting the paramedian pontine reticular formation pprf, abducens nucleus. 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.
Eightandahalf syndrome american academy of ophthalmology. 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. 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 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. In one case the oneandahalf syndrome evolved into a total horizontal gaze palsy, which corresponded to involvement of the abducens nucleus contralateral to the initially destroyed paramedian. 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. Pdf oneandahalf syndrome is characterized by combination of the clinical features of unilateral horizontal gaze palsy and.
One and onehalf syndrome with supranuclear facial weakness. Intelligence and life expectancy are not usually affected. A 36yearold woman presented with left gaze limitation and right eye limitation of adduction with horizontal nystagmus on abduction oneandahalf syndrome in addition to a leftsided lower motor neuron facial nerve palsy figure 1 and video. One anda half syndrome is a gaze abnormality characterized by a conjugate horizontal gaze palsy in one direction plus an internuclear ophthalmoplegia in the other. Stevensjohnson syndrome and toxic epidermal necrolysis are known to be lifethreatening mucocutaneous reactions, the mortality rates for which are as high as 30%.
The mlf connects the abducens nerve on one side with the occulomotor nerve on the contralateral side. The syndrome is usually due to a single unilateral lesion of the paramedian pontine reticular formation or the abducens nucleus on one side causing the conjugate gaze palsy, with interruption of. Botulinum toxin treatment of one and a half syndrome. Diabetes mellitus can be presented rarely with one and half syndrome being the first presentation. The one andahalf syndrome9 oahs is an ocular movement disorder characterized by lateral gaze palsy to one direction associated with internuclear. The only remaining horizontal movement is contralateral abduction. A one and a half syndrome involves lesions of ipsilateral pprf, or abducens, along with ipsilateral mlf. Ocular motor examination revealed combination of right gaze paresis and right internuclear ophthalmoplegia suggestive of horizontal oneandahalf syndrome fig 1ac.
A one an d a half synd rome involves lesions of ipsilateral pprf, or abducens, along with ipsilateral mlf. Facial nucleusfascicles in the lower pontine tegmentum contributed to the observed clinical signs. 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. Stevensjohnson syndrome and toxic epidermal necrolysis are one of the few dermatological diseases that constitute a medical emergency. Oneandahalf syndrome is a disorder of horizontal ocular movement characterized by a lateral gaze. A 12yearold girl with eczema not responding to treatment.
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. Fishers one and a half syndrome eccles health sciences. 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. Three cases of isolated oneandahalf syndrome with facial nerve palsy related to infarction are presented. Pdf one and onehalf syndrome with supranuclear facial. 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. Magnetic resonance imaging revealed an area of hyperintense signal extending beyond and lateral to the paramedian pontine territory and ventrally to. A clinical diagnosis of one and a half syndrome secondary to a cerebrovascular event was made. Oneandahalf 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.
Sixteen syndromea new pontine ophthalmoneurological syndrome within the oneandahalf syndrome spectrum of disorders. Diabetic third nerve palsies may be caused by ischemia in midbrain or along peripheral nerve sparing the. The oneandahalf syndrome is a term originally proposed by fisher to describe a syndrome with ocular dyskinesia, exhibiting ipsilateral conjugate gaze palsy and ipsilateral internuclear ophthalmoplegia ino. Horizontal gaze palsy in oneandahalf syndrome may result from lesions in the abducens nucleus, in the abducens nerve and the medial longitudinal fasciculus, or in the pontine paramedian reticular formation pprf. One and a half syndrome complicating resection of fourth. Pdf the oneandahalf syndromea unilateral disorder of the.
The two constituents of the oneandahalf syndrome ipsilateral horizontal gaze palsy and ino can be analyzed anatomically to provide the basis for topographic localization of the lesion at the bedside. Supranuclear ocular movements comprise chiefly vertical and horizontal movements. 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. This case talks about oneandahalf plus syndrome, a clinical syndrome affecting binocular vision and facial nerve. Oneandahalf syndrome is characterized by conjugate horizontal gaze palsy in one direction in addition to contraversive internuclear ophthalmoplegia.
Living life to the fullest with ehlersdanlos syndrome pdf. This ms patient had fishers oneandahalf syndrome characterized by. In one case the one an dahalf synd rome evolved into a total horizontal gaze palsy, which corresponded to involvement of the abducens nucleus contralateral to the initially destroyed paramedian. The treatment depends on the causes of the syndrome.
Isolated pprf lesions are associated with retained. Botulinum toxin treatment of one and a half syndrome pdf. Since neither of these structures was within the area of the cysts, it is puzzling as to why they would have been affected by. There is also interruption of internuclear fibres of.
Frcp harvard medical school overview of tdp43 proteinopathy. Oneandahalf 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. 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. 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 is characterized by conjugate horizontal gaze palsy in. 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. Vertical and horizontal one and a half syndrome with. We describe a patient with one and a half syndrome without any other brainstem signs, caused by neurocysticercosis involving the pons. When last examined 2 months after the onset of symptoms, the patient showed only a minimal deficit in right horizontal gaze. Diabetes mellitus presenting as one and half syndrome. Faqs download template researchers guide article pattern. Pdf the oneandahalf syndrome is a clinical disorder of extraocular movements characterized by a conjugate. 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.
People with this condition may also have distinctive facial features, extra folds of skin on the neck, and short stature. I am one of those people who have driven outofstate to. Like horizontal one and a half syndrome, vertical one and a half syndrome represents an important neurological signs suggestive of a lesion affecting the rostral midbrain. Sixteen syndromea new pontine ophthalmoneurological. To provide clinicoanatomical correlation for a small pontine tegmental ischemic stroke producing the one and onehalf syndrome associated with supranuclear facial weakness.
The article is mainly to summarize and analyse the clinical features, pathological anatomy. Illustration of lesions in one and a half syndrome. Neurologic problems persist in up to 20 percent of patients with the disease, and onehalf of these patients are severely disabled. Mri showed multiple periventricular white matter lesions and a central lesion in the pontine tegmentum figure. Oneandahalf syndrome is a gaze abnormality characterized by a conjugate horizontal gaze palsy in one direction plus an internuclear ophthalmoplegia in the other. The one and a half syndrome is a rare weakness in eye movement affecting both eyes, in which. Oneandahalf syndrome with its spectrum disorders ncbi. Oneandahalf syndrome is a clinical disorder characterized by an ipsilateral conjugate horizontal gaze palsy the one and an ipsilateral internuclear ophthalmoplegia ino the half. 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.
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