More formally, it is characterized by a conjugate horizontal gaze palsy in one direction and an internuclear ophthalmoplegia in the other. 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. Eightandahalf syndrome american academy of ophthalmology. Diabetes mellitus presenting as one and half syndrome.
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. The mlf connects the abducens nerve on one side with the occulomotor nerve on the contralateral side. One and onehalf syndrome with supranuclear facial weakness. 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. 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.
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. A one an d a half synd rome involves lesions of ipsilateral pprf, or abducens, along with ipsilateral mlf. The only remaining horizontal movement is contralateral abduction. Judged clinically the patient made a nearly complete recovery, but little change was apparent in the nmr image six months later. One and a half syndrome an overview sciencedirect topics. Vertical oneandahalf syndrome clinical, oculography and. Bennett lecture american neurologic association 2008. 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 one and a half syndrome involves lesions of ipsilateral pprf, or abducens, along with ipsilateral mlf.
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. 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. 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. This syndrome is typically related with damage to the paramedian pontine reticular formation pprf and the medial longitudinal fasciculus mlf on the same side. Magnetic resonance imaging in cases 1 and 2 was unremarkable, whereas magnetic resonance angiography demonstrated pathophysiologically significant vertebral basilar disease. Shared mechanisms underlying frontotemporal lobar degeneration and amyotrophic lateral sclerosis the f. Ocular motor examination revealed combination of right gaze paresis and right internuclear ophthalmoplegia suggestive of horizontal oneandahalf syndrome fig 1ac. 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. 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. A 12yearold girl with eczema not responding to treatment. 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.
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. Pdf the oneandahalf syndromea unilateral disorder of the. Sixteen syndromea new pontine ophthalmoneurological syndrome within the oneandahalf syndrome spectrum of disorders. 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. 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. The patient with horizontal gaze palsy typically is unable to move either eye beyond the. Vertical and horizontal one and a half syndrome with. 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. The underlying pathological lesion is frequently at the level of the unilateral tegmentum, affecting the paramedian pontine reticular formation pprf, abducens nucleus. This ms patient had fishers oneandahalf syndrome characterized by.
Neurologic problems persist in up to 20 percent of patients with the disease, and onehalf of these patients are severely disabled. 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. Living life to the fullest with ehlersdanlos syndrome 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, and comment on the horizontal gaze physiology. Stevensjohnson syndrome and toxic epidermal necrolysis are known to be lifethreatening mucocutaneous reactions, the mortality rates for which are as high as 30%. 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. 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. Living life to the fullest with ehlersdanlos syndrome pdf free download. Sixteen syndromea new pontine ophthalmoneurological. Botulinum toxin treatment of one and a half syndrome pdf.
Fishers one and a half syndrome eccles health sciences. 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. Frcp harvard medical school overview of tdp43 proteinopathy. 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. Oneandahalf syndrome is a disorder of horizontal ocular movement characterized by a lateral gaze. Supranuclear ocular movements comprise chiefly vertical and horizontal movements. Conceptual medicine by dr prathap bingi 11,059 views. 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. Faqs download template researchers guide article pattern. The exact anatomical and physiological mechanism underlying vertical gaze still remains obscure. 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. 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.
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. 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. Supranuclear downgaze paralysis with monocular elevation palsy, better known as vertical oneandahalf syndrome, results from lesions affecting the mesodiencephalic region. Facial nucleusfascicles in the lower pontine tegmentum contributed to the observed clinical signs. One and a half syndrome complicating resection of fourth. Isolated pprf lesions are associated with retained. We describe a patient with one and a half syndrome without any other brainstem signs, caused by neurocysticercosis involving the pons.
Intelligence and life expectancy are not usually affected. Diabetes mellitus can be presented rarely with one and half syndrome being the first presentation. Stevensjohnson syndrome and toxic epidermal necrolysis are one of the few dermatological diseases that constitute a medical emergency. Sheldonhall syndrome, also known as distal arthrogryposis type 2b, is characterized by joint deformities contractures that restrict movement in the hands and feet. The one andahalf syndrome9 oahs is an ocular movement disorder characterized by lateral gaze palsy to one direction associated with internuclear. I am one of those people who have driven outofstate to. Oneandahalf syndrome is a clinical disorder characterized by an ipsilateral conjugate horizontal gaze palsy the one and an ipsilateral internuclear ophthalmoplegia ino the half. 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 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. People with this condition may also have distinctive facial features, extra folds of skin on the neck, and short stature. Send us your ophthalmic image and its explanation in 150250 words. Botulinum toxin treatment of one and a half syndrome. 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. 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.
We believe that our patients weakness resulted from involvement of the fourth bundle of dejerine in the pontine tegmentum. The article is mainly to summarize and analyse the clinical features, pathological anatomy. A 58yearold africanamerican woman presented with blurry vision and foreign body sensation in the left eye. When last examined 2 months after the onset of symptoms, the patient showed only a minimal deficit in right horizontal gaze. To provide clinicoanatomical correlation for a small pontine tegmental ischemic stroke producing the one and onehalf syndrome associated with supranuclear facial weakness. Three cases of isolated oneandahalf syndrome with facial nerve palsy related to infarction are presented. Pdf the oneandahalf syndrome is a clinical disorder of extraocular movements characterized by a conjugate. 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. The treatment depends on the causes of the syndrome. There is also interruption of internuclear fibres of. Illustration of lesions in one and a half syndrome. Oneandahalf syndrome is characterized by conjugate horizontal gaze palsy in. This case talks about oneandahalf plus syndrome, a clinical syndrome affecting binocular vision and facial nerve. On my good days, the sacrum holds and the up slips have gone away. 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.
The one and a half syndrome is a rare weakness in eye movement affecting both eyes, in which. Diplopia 12 blurred vision 8 oscillopsia 4 difficulty looking to one side 2 quivering of the eye 1 no visual complaint 3 table 2. Magnetic resonance imaging revealed an area of hyperintense signal extending beyond and lateral to the paramedian pontine territory and ventrally to. Oneandahalf syndrome american academy of ophthalmology. Oneandahalf syndrome is a gaze abnormality characterized by a conjugate horizontal gaze palsy in one direction plus an internuclear ophthalmoplegia in the other. Pdf oneandahalf syndrome is characterized by combination of the clinical features of unilateral horizontal gaze palsy and. Oneandahalf syndrome is characterized by conjugate horizontal gaze palsy in one direction in addition to contraversive internuclear ophthalmoplegia. Diabetic third nerve palsies may be caused by ischemia in midbrain or along peripheral nerve sparing the. Oneandahalf syndrome with its spectrum disorders ncbi. Mri showed multiple periventricular white matter lesions and a central lesion in the pontine tegmentum figure. A clinical diagnosis of one and a half syndrome secondary to a cerebrovascular event was made.
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