Prevalence and Improvement of Caine-Positive Wernicke-Korsakoff Syndrome in Psychiatric Inpatient Admissions.

BACKGROUND: Wernicke-Korsakoff Syndrome (WKS) resulting from thiamine deficiency is classically defined as including encephalopathy, ataxia, and ophthalmoplegia. Only 16% of autopsy-confirmed patients with WKS exhibit all three signs. Caine-positive WKS criteria include two or more of the following: nutritional deficiency, delirium or mild memory impairment, cerebellar dysfunction/ataxia, and oculomotor abnormalities. OBJECTIVE: We describe Caine-positive WKS prevalence among psychiatric inpatients and compare pretreatment-versus-posttreatment neurocognitive improvement to an unaffected group. METHODS: This 6-month quality-improvement evaluation included two-stage screening for Caine-positive WKS, administering high-dose intravenous thiamine (day 1: 1200 mg; days 2-4: 200 mg) with reexamination on day 5. We used descriptive statistics and fitted random effects models to examine rate-of-change differences in pre-/posttreatment Montreal Cognitive Assessment (MoCA), delayed 5-item recall, and gait/coordination scores between treated Caine-positive patients with WKS and untreated Caine-negative patients. RESULTS: Of 262 patients, 32 (12%) had Caine-positive WKS; 17 (53%) used alcohol currently. Treated Caine-positive WKS (n = 26) versus Caine-negative comparison (n = 34) before and after treatment observed a mean change (standard deviation) in the MoCA score of 3.6 (2.5) versus 1.8 (2.5) (P < 0.01); 5-item recall: 1.8 (1.4) versus 0.5 (1.4) (P < 0.001); gait/coordination scores: -0.6 (1.2) versus -0.1 (0.6) (P < 0.001). Oculomotor abnormalities were infrequent (n = 4 in Caine-positive WKS, n = 2 in Caine-negative comparison groups). CONCLUSIONS: Caine-positive WKS prevalence among psychiatric inpatients was 12%; only half used alcohol. Patients treated with high-dose thiamine demonstrated clinically significant neurocognitive improvement.

[Staged complex treatment of paralytic lagophthalmos (case report)]. / Kompleksnoe poetapnoe lechenie paraliticheskogo lagoftal'ma (klinicheskii sluchai).

Paralytic lagophthalmos, resulting from facial nerve palsy, is a difficult medical and social issue that requires cooperation of different specialists. Complications that arise in paralytic lagophthalmos may cause significant vision loss and even eye loss. Various techniques of paralytic lagophthalmos correction are used to protect the cornea and restore eyelid anatomy and functions. These comprise palliative (conservative), surgical, and alternative treatments (such as botulinum toxin type A therapy). Surgical treatment of paralytic lagophthalmos patients often has to be staged and complex. This article presents a clinical case of a female patient with paralytic lagophthalmos complicated by corneal perforation. Her staged complex treatment included lower eyelid surgery, chemodenervation of the upper eyelid levator and optical reconstructive surgery. The following positive results were achieved: the protective function of the eyelids was restored, residual visual functions - preserved, the risk of eye loss - eliminated, and the asymmetry between the two halves of the face - corrected.

Impact of miglustat on evolution of atypical presentation of late-infantile-onset Niemann-Pick disease type C with early cognitive impairment, behavioral dysfunction, epilepsy, ophthalmoplegia, and cerebellar involvement: a case report.

BACKGROUND: Niemann-Pick disease type C is a rare inherited neurodegenerative disease involving impaired intracellular lipid trafficking and accumulation of glycolipids in various tissues, including the brain. Miglustat, a reversible inhibitor of glucosylceramide synthase, has been shown to be effective in the treatment of progressive neurological manifestations in pediatric and adult patients with Niemann-Pick disease type C, and has been used in that indication in Europe since 2010. CASE PRESENTATION: We describe the case of a 16-year-old white French boy with late-infantile-onset Niemann-Pick disease type C who had the unusual presentation of early-onset behavioral disturbance and learning difficulties (aged 5) alongside epileptic seizures. Over time he developed characteristic, progressive vertical ophthalmoplegia, ataxic gait, and cerebellar syndrome; at age 10 he was diagnosed as having Niemann-Pick disease type C based on filipin staining and genetic analysis (heterozygous I1061T/R934X NPC1 mutations). He was commenced on miglustat therapy aged 11 and over the course of approximately 3 years he showed a global improvement as well as improved cognitive and ambulatory function. During this period he remained seizure free on antiepileptic therapy, using valproate and lamotrigine. CONCLUSIONS: Miglustat improved the neurological status of our patient, including seizure control. Based on our findings in this patient and previous published data, we discuss the importance of effective seizure control in neurological improvement in Niemann-Pick disease type C, and the relevance of cerebellar involvement as a possible link between these clinical phenomena. Thus the therapeutic efficacy of miglustat could be hypothesized as a substrate reduction effect on Purkinje cells.

Consecuencia del abuso de alcohol / A consequence of alcohol abuse

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Dynamic changes in sleep-related breathing abnormalities in bilateral paramedian mesencephalon and thalamus stroke: a follow-up case study.

BACKGROUND: Bilateral paramedian thalamic stroke is characterized by hypersomnia, vertical gaze palsy, amnestic alteration, and apathic state. Combined lesion of the paramedian thalamus and mesencephalon bilaterally is extremely rare. Little is known about the breathing disturbances of the particular region due to the lesion. The following describes the specific case of a woman, age 62, with bilateral paramedian thalamic and mesencephalic stroke. Initially, the patient's complaints exhibited altered vigilance and vertical gaze palsy. Notably, following the acute phase, fluctuating hypersomnia was detected. The MRI (brain) revealed an ischemic lesion in the medial part of the mesencephalon and paramedian thalamus, bilaterally. AIMS: The aim of the present study is to elucidate the involvement and characteristics of sleep-related breathing abnormalities in the clinical manifestation of the combined paramedian thalamic and mesencephalic stroke. METHODS: Polysomnographic recordings were accomplished seven times with 1-week interval between the consecutive recordings, toward investigating the early changes of sleep and sleep-related breathing abnormalities. RESULTS: Sleep structure examination featured a decrease in N3 and REM ratio and an increase in N1 and N2 ratio with minimal improvement during the recovery period. In contrast, significant changes were found in the breathing pattern: the initial central apnea dominance was followed by obstructive apneas with a gradual decrease of the total pathological respiratory events. CONCLUSION: In addition to the structural abnormality of the sleep regulating network, sleep-disordered breathing is another possible cause of hypersomnia in patients afflicted with the present localization of the lesion.

[Ophthalmic complications and local anesthesia. Pathophysiology and types of eye complications after intraoral dental anesthesia, and clinical recommendations]. / Ophthalmologische Komplikationen und Lokalanästhesie. Pathophysiologie und Formen der Augenkomplikationen nach intraoraler zahnärztlicher Lokalanästhesie und klinische Empfehlungen.

The present article reviews the different types of ophthalmologic complications following administration of intraoral local anesthesia. Since the first report by Brain in 1936, case reports about that topic have been published regularly in the literature. However, clinical studies evaluating the incidence of ophthalmologic complications after intraoral local anesthesia are rarely available. Previous data point to a frequency ranging from 0.03% to 0.13%. The most frequently described ophthalmologic complications include diplopia (double vision), ptosis (drooping of upper eyelid), and mydriasis (dilatation of pupil). Disorders that rather affect periorbital structures than the eye directly include facial paralysis and periorbital blanching (angiospasm). Diverse pathophysiologic mechanisms and causes have been reported in the literature, with the inadvertent intravascular administration of the local anesthetic considered the primary reason. The agent as well as the vasopressor is transported retrogradely via arteries or veins to the orbit or to periorbital structures (such as the cavernous sinus) with subsequent anesthesia of nerves and paralysis of muscles distant from the oral cavity. In general the ophthalmologic complications begin shortly after administration of the local anesthesia, and disappear once the local anesthesia has subsided.

Transient impaired vision, external ophthalmoplegia, and internal ophthalmoplegia after blepharoplasty under local anesthesia.

PURPOSE: To report a case of transient bilateral vision impairment with external ophthalmoplegia and internal ophthalmoplegia after blepharoplasty under local anesthesia. DESIGN: Interventional case report. METHODS: A 70-year-old man underwent bilateral upper blepharoplasty under local anesthesia. During orbital fat removal additional anesthetic was injected into both medial fat pads for pain control. RESULTS: Immediate postoperative examination revealed bilateral decreased visual acuity and internal ophthalmoplegia in the right eye. An exotropia was present with marked limitation of right eye adduction. These findings resolved completely 3 hours postoperatively. CONCLUSIONS: Local anesthesia during blepharoplasty can enervate the optic nerve, ciliary ganglion, and extraocular muscle nerves. Local anesthesia should be injected judiciously during orbital fat removal to avoid this reversible but alarming event.

Fascicular arrangement within the oculomotor nerve MRI analysis of a midbrain infarct.

The fascicular arrangement of the oculomotor nerve within the midbrain is not adequately elucidated in humans. We treated a patient with a partial oculomotor palsy who had impaired adduction and supraduction on the left side, which were attributed to an ipsilateral lacunar infarct. CT and MRI revealed a discrete lesion in the centre of the midbrain tegmentum in the rostrocaudal plane. This case suggests that the oculomotor fibres for extraocular movement are located in the middle of the the midbrain, and supports the fascicular proximity of the superior and medial rectus muscles. The fascicular arrangement of the midbrain oculomotor nerve is speculated to be pupillary component, extraocular movement and eyelid elevation in that rostrocaudal order, based on the previous reports of neuro-ophthalmological impairment and MRI findings, which are analogous to the nuclear arrangement proposed by Warwick.

Superior oblique muscle paresis after sub-Tenon's anesthesia for cataract surgery.

A 68-year-old man developed strabismus after having sub-Tenon's anesthesia for cataract extraction and intraocular lens implantation. An ipsilateral hypertropia with superior oblique muscle paresis developed in the operated eye. The hypertropia appeared 1 day after surgery and resolved 1 month later. Although sub-Tenon's anesthesia is considered safer than other methods of local anesthesia, strabismus may occur.

Restoration of function in the paralyzed rabbit orbicularis oculi muscle by direct functional electrical stimulation.

Restoration of the ability to blink and protect the eye in the patient with facial paralysis remains a challenge. Although many treatments exist, no one approach corrects all the deficits associated with the loss of orbicularis oculi function. In this study, the author investigated the feasibility of restoring function by direct electrical stimulation of the paralyzed orbicularis oculi muscle in the rabbit model. Using a pacing device developed by the author, functional restoration of a normal-appearing blink was produced throughout 30 days of continuous pacing in six rabbits with transected facial nerves. Histologic evaluations of the paced tissues demonstrated no evidence of detrimental effects attributable to the electrical stimulation. The findings of this study support the feasibility of employing direct electrical stimulation to restore the function of paralyzed orbicularis oculi muscles. Potential applications may also exist in other areas in which peripheral denervation creates functional impairment.

[Selective impairment of downward gaze; report of two cases of midbrain and bilateral paramedian thalamic infarction].

Selective paralysis of downward gaze, first described by André-Thomas et al. in 1933, is rare to be observed and not many cases have been so far reported in the literature. Two additional cases of selective impairment of downward gaze have been reported. X-ray CT scan revealed the infarcted areas in the midbrain tegmentum and bilateral paramedian thalami in both cases. Angiographic studies have revealed that the arteries occluded might be the paramedian thalamic arteries and their mesencephalic branches. According to the reported clinico-pathological studies and, in addition, to the results of the physiological studies in animal models, the responsible site of lesion for downward gaze failure is thought to be the rostral interstitial nucleus of MLF (riMLF). In one of the cases (28 year-old female, case 1), while vertical slow pursuit eye movements and upward saccade were normal in speed and range, maximum speed of eyes in voluntary downward saccade was markedly reduced and the eyes moved down slowly as if moving in oil (slow eye movement, viscosité). Passively extending the patient's head while she tried to fix her eyes on an unmoving object (doll's eye phenomenon), the speed of her eyes in downward saccade did not increase. In the other case (45 year-old male, case 2), the patient's voluntary and reflex downward eye movements were completely impaired and he could not look down nor follow the object below extending the horizontal meridian level. With regard to the unique features of abnormal eye movements observed in our cases, the possibility of coexistence of saccadic and slow pursuit eye movement systems in the vertical plane was discussed.

[Anesthesia for eye operations in mitochondrial encephalomyelopathy]. / Anästhesie für Augenoperationen bei mitochondrialer Enzephalomyopathie.

Mitochondrial encephalomyopathy involves a disturbance of the mitochondrial respiratory chain, as a result of which the blood lactate level is elevated. In stress situations a lactate acidosis can occur. The disease may be subdivided into three main syndromes: Kearns-Sayre syndrome (KKS), "myoclonus epilepsy with ragged red fibers syndrome" (MERRF), and "mitochondrial myopathy, encephalopathy, lactic acidosis and strokelike episodes syndrome" (MELAS). There are also several intermediate forms. Ophthalmological symptoms are frequent and occasionally have to be treated surgically. A 20-year-old male patient with a mixed form of these syndromes including elements of KSS and MERRF had to undergo cataract extraction. The authors decided to perform the operation under local anesthesia and sedation, with the anesthetist on standby. No problems arose. In all cases where mitochondrial encephalomyopathy is suspected the diagnosis should be confirmed by a muscle biopsy and the risk of cardiac arrest, respiratory insufficiency, and epileptic seizures ruled out prior to surgery. Local anesthesia with sedation appears to be the most favorable form of anesthesia provided the maximum dose is observed and a substance with a high convulsion threshold is chosen. Perioperative monitoring by an anesthetist and temporary provision of a cardiac pacemaker are necessary.(ABSTRACT TRUNCATED AT 250 WORDS)

Vertical gaze ophthalmoplegia from infarction in the area of the posterior thalamo-subthalamic paramedian artery.

Three cases of a stroke syndrome of acute supranuclear vertical gaze ophthalmoplegia are presented. Voluntary vertical gaze and the vertical vestibulo-ocular reflex were absent or diminished initially in all three patients. The patients also had loss of convergence and alteration in their mental status. Computed tomography scans showed lesions in the region of the rostral midbrain and lower diencephalon in the area surrounding the third ventricle. The blood supply, ischemic stroke syndrome and function of this region with reference to the supranuclear control of vertical gaze are discussed.

Contralateral decreased visual acuity and extraocular muscle palsies following retrobulbar anesthesia.

Numerous complications resulting from retrobulbar injections in the injected eye and orbit have been reported. A rare complication of retrobulbar anesthesia is the occurrence of decreased visual acuity and extraocular muscle palsies in the contralateral eye. We report three cases of contralateral dysfunction of cranial nerves II and III, following retrobulbar anesthesia. None of the patients suffered permanent sequelae. Several methods of decreasing the probability of such an occurrence are discussed.

Parinaud's syndrome: electro-oculographic and anatomical analyses of six vascular cases with deductions about vertical gaze organization in the premotor structures.

Six cases of Parinaud's syndrome, with downward (Cases 1, 2), upward (Cases 3, 4) and both downward and upward gaze paralysis (cases 5, 6) are reported. Four cases (Cases 1, 2, 3, 5) were studied anatomically using serial sections of the brain and 3 cases (Cases, 1, 4, 6) analysed electro-oculographically. In all the cases there were rather small vascular lesions in the mesodiencephalic region, sparing the oculomotor nuclei. Since the rostral interstitial nuclei of the medial longitudinal fasciculus (riMLF), located above the oculomotor nuclei, contain the final relays producing all vertical saccades, it is suggested that the different aspects of Parinaud's syndrome may result from damage to their cells or to their excitatory efferent tracts, or even to their afferent pathways. Downgaze paralysis results from bilateral lesions involving the regions located just caudal, medial and dorsal to the upper poles of the red nuclei. The critical area is probably related to the mediocaudal part of the riMLF, the lateral portion of which appears to be spared. These anatomical data, combined with the clinical observation that most downward eye movements (except slow reflex movements) are affected in the case with such paralysis, lead us to propose that it is the riMLF efferent tracts mediating downgaze and projecting on to the oculomotor nuclei that are principally damaged by the lesions. Upgaze paralysis results from unilateral lesions in or near the posterior commissure. The clinical data allow us to propose that it is also the riMLF efferent tracts, mediating upgaze, that are damaged in such cases. consequently these tracts, probably originating from the dorsolateral part of the riMLF, would decussate through the posterior commissure before they reach the oculomotor nuclei. Combined downgaze and upgaze paralysis results from bilateral lesions involving the region related to the whole riMLF on both sides. The principal conclusion is that the riMLF efferent tracts mediating upward and downward gaze have clearly separate courses in the immediate premotor structures.

The 'subcortical dementia' of progressive supranuclear palsy.

Progressive supranuclear palsy (Steele et al.) has a characteristic pattern of dementia: (1) forgetfulness, (2) slowing of thought processes, (3) emotional or personality changes (apathy or depression with occasional outbursts of irritability), and (4) impaired ability to manipulate acquired knowledge. In many neurological disease states associated with subcortical pathology a similar pattern of dementia exists. The neurobehavioural changes of progressive supranuclear palsy thus typify a clinical pattern which may be referred to as subcortical dementia. The subcortical dementias have a striking clinical resemblance to the dementia which occurs after bifrontal lobe disease. However, the subcortical dementias can be clearly distinguished clinically from cortical dementias, other than frontal dementias. We propose as a tentative hypothesis that there may be common pathophysiological mechanisms underlying the subcortical dementias-in particular, disturbances of timing and activation. There are immediate practical implications of this hypothesis: drugs which have an effect on subcortical timing and activating mechanisms may be useful in the treatment of subcortical dementias.