Abduction Deficit Research Articles

蝶形骨洞炎の先行感染が示唆されたＦｉｓｈｅｒ症候群

Fisher syndrome is a subtype of Guillain-Barré syndrome that is characterized by the three chief symptoms of acute-onset external ophthalmoplegia, ataxia, and loss of tendon reflexes. Herein, we report a case of Fisher syndrome encountered by us,with sphenoiditis as the antecedent infection. The patient was a 39-year-old man who visited a local doctor after developing the symptom of rhinorrhea, followed three days later by diplopia. Cranial MRI suggested sphenoid sinusitis, and the patient was referred to our hospital with suspected rhinogenous intraorbital complication. Neurological findings included bilateral abduction deficit, which was considered unlikely to be an ocular manifestation of an intraorbital complication of right unilateral sphenoiditis. In addition, the reduction of the tendon reflexes in the limbs and ataxia suggested the diagnosis of Fisher syndrome, which was then confirmed based on the albuminocytologic dissociation observed on cerebrospinal fluid examination and a positive blood test result for anti-GQ1b antibody. The symptoms were attributed to the production of antibodies against antigens expressed on the oculomotor, trochlear, abducens and other nerves (anti-GQ1b antibody) due to the antecedent infection. The present patient was thought to have developed Fisher syndrome following sphenoiditis as an antecedent infection. Care must be taken to differentiate this condition from rhinogenous intraorbital complication.

Bilateral Abducens Palsies and Facial Weakness as Initial Manifestations of a Chiari 1 Malformation

Chiari I malformations are rare, congenital anomalies involving the caudal herniation of the cerebellar tonsils into the upper cervical spinal canal. Osseous abnormalities of the skull permit the extension of the hindbrain below the foramen magnum and engender syringohydromyelia formation. Neuroophthalmic manifestations are not uncommon; nevertheless, this is the first report of concomitant bilateral abducens palsies and facial nerve impairment as presenting manifestations of a Chiari I malformation. A 30-year-old black female presented with a recent history of blurred vision and binocular diplopia in both left and right gazes. Headaches, dizziness, and orofacial sensorimotor impairments were noted in conjunction with the onset of her diplopia. No history of surgery, trauma, or systemic illness was elicited. Extraocular motility testing confirmed a complete bilateral abduction deficit. Additional cranial nerve testing revealed bilateral facial nerve weakness and tactile hypesthesia in the perioral region. No taste disturbances were reported. Nuclear magnetic resonance imaging studies revealed a Chiari I malformation with a syringohydromyelia formation in the vicinity of the patient's sixth cervical vertebrae. Foramen magnum decompression surgery was performed. Two months after surgery, abduction capacity was restored along with resolution of symptoms of perioral numbness and signs of facial weakness. Bilateral sixth nerve palsies are rare clinical commodities especially when they do not occur in isolation. Intracranial neoplams, trauma, subarachnoid hemorrhaging, demyelinating disease, and meningeal infarcts have all been shown to induce mixed cranial neuropathies. The concomitant bilateral abducens palsy and concomitant facial nerve weakness reported in this case represents an atypical and previously unreported presentation of a Chiari I malformation.

Reconstruction after Chronic Extensor Pollicis Longus Ruptures

Different types of secondary reconstruction of extensor pollicis longus tendon injuries have been reported, with repair using the extensor indicis proprius being the gold standard. This technique, however, may decrease extension strength and independent extension of the index finger. We presumed repair with the accessory abductor pollicis longus would achieve functional motion of the thumb and avoid donor site morbidity. We retrospectively report the results for 11 consecutive patients who had repair with the accessory abductor pollicis longus and were followed prospectively for a minimum of 2 years. Study-specific outcome measures were used to assess function in activities of daily living, pain, and patient satisfaction. According to the score described by Geld-macher et al, good or excellent results were achieved in all cases. Mean abduction deficit of the thumb was 10 degrees compared with the uninjured side. Using a 0- to 10-point visual analog scale self-reporting questionnaire, patients obtained mean results of 7.9 points for pain, 7.5 points for activities of daily living, and 7.6 points for patient satisfaction. We recommend the accessory abductor pollicis longus as an alternative for extensor pollicis longus tendon repair.

Bilateral familial Duane’s retraction syndrome

Background Duane’s retraction syndrome (DRS) is a congenital oculomotor anomaly that is accompany by globe retraction with simultaneous narrowing of the palpebral fissure on attempted adduction. Hereditary factors have been postulated to be associated with the development of DRS since 1879. However, the modern literature fails to provide substantial scientific evidence to support this premise. Case report A 7-year-old patient presented with bilateral abduction deficit and retraction of the globe with narrowing of the palpebral fissure on attempted adduction. Conclusion We present a case of Duane’s retraction syndrome in which an investigation of the family history uncovered 4 generations of affected family members, lending support to the theory that some cases of DRS are inherited.

Ocular manifestations in Kabuki syndrome: the first report from Saudi Arabia

Kabuki syndrome (KS) is a multiple congenital anomaly syndrome in which ophthalmological examination for the early detection of ocular abnormalities is desired in order to prevent visual impairment. Retrospective, interventional, case report of a 5-year-old female patient of Arabic origin with features of Kabuki syndrome. Patient had neurological deficit, psychomotor retardation, a peculiar face, including large prominent cup shaped ears, broad depressed nasal tip, and high arched palate, and malformed teeth. Her ocular features suggestive of Kabuki syndrome included left upper eyelid congenital ptosis, lagophthalmos, arched eyebrows with temporal sparing of hair, long horizontal palpebral fissures, lateral lower eyelid eversion and resultant epiphora. Other abnormalities included medial lower epicanthal folds, abduction deficit bilaterally, large esotropia, significant hyperopia, right corneal opacity, iris and chorioretinal coloboma. Patient required hyperopic correction and ptosis surgery, which improved her visual functioning. We report the first case of a Kabuki syndrome patient from Saudi Arabia and stress on the importance of ophthalmological examination in all patients with KS for the early detection of ocular anomalies in order to prevent visual impairment.

Surgical Strategies for Brachial Plexus Polio-Like Paralysis

Brachial plexus polio-like paralysis is an acute flaccid paralysis of the upper limb following viral infection. Surgical approaches to the paralytic limb have not previously been discussed in the literature. This study is the first to describe the clinical course and propose a surgical strategy for disabilities related to poliomyelitis-like paralysis. Between 1982 and 2004, 17 patients (11 boys and six girls) presented with acute flaccid paralysis of the upper limb. Their clinical course was reviewed retrospectively. Average age at onset of disease was 2 years (range, 4 months to 13 years). All patients had disability in shoulder abduction and/or elbow flexion. Ten patients underwent reconstructive surgery. Stage V shoulder abduction (>160 degrees) according to Gilbert's classification was regained in five patients who underwent nerve transfer within 1 year of paralysis. One patient treated by nerve transfer after 3 years of paralysis obtained only stage I abduction (<45 degrees). In two patients, multiple local muscle transfers were performed for the shoulder abduction disability, resulting in mild improvement from stage I to stage II abduction (<90 degrees). In four patients, functioning free muscle transplantation for elbow flexion was carried out, and all regained functional M4 muscle strength. Of seven patients in the nonsurgical group, two had complete spontaneous recovery within 1 year, but five had permanent residual limb paralysis at a mean follow-up of 10 years. Surgical strategies, including nerve transfer for shoulder abduction deficit within 1 year after attack and functioning free muscle transplantation for the elbow flexion deficit in the late period, should be considered for this disease. Late reconstruction, either by nerve transfer or by using local multiple muscle transfer for shoulder abduction, is ineffective.

Sudden bilateral blindness in Wernicke's encephalopathy: Case report and review of the literature

We report on a patient suffering from bilateral sudden blindness as initial symptom of Wernicke's encephalopathy (WE). A 37-year-old male alcoholic was admitted to a psychiatric clinic because of excessive alcohol consumption (3.4‰). 24 h later he developed acute bilateral blindness with no light perception, downbeat nystagmus, bilateral ocular abduction deficits, cerebellar ataxia as well as a slight psychomotor slowing and mild disorientation. MRI including diffusion-weighted imaging and MR-angiography 3 h after symptom onset did not reveal findings suggestive for ischemic stroke. Immediate iv-application of thiamine led to a nearly complete remission of the neuroophthalmologic symptoms within 12 h. Although we critically discuss other potential etiologies, we conclude that the complex clinical picture with initial sudden blindness is an unusual presentation of WE.

Prostate cancer metastasis to clivus causing cranial nerve VI palsy

Background An abduction deficit can have many potential etiologies. Clinical testing can help distinguish a neurogenic from a restrictive process. For any patient with a current or past history of cancer, even in the setting of vasculopathic risk factors, a further workup is necessary to rule out a metastatic process. Case report A 66-year-old man reported sudden blurry vision but did not describe a definite diplopia. Clinical evaluation found left cranial nerve (CN) VI palsy. Although he did have vasculopathic risk factors, neuroimaging found prostate cancer metastasis to the mid to left clivus, extending to the left cavernous sinus region as well as a smaller metastasis to the left temporal lobe. The patient underwent radiation treatment with improvement in his clinical presentation and symptoms. His prostate cancer was subsequently treated more aggressively, and 2 years later, despite spinal metastases, he was doing relatively well. Conclusion Prostate cancer commonly metastasizes, with a high propensity to invade bone. CN VI runs along the midline-structured bony clivus, between the pons and the cavernous sinuses. Therefore, a metastatic lesion to the clivus can be responsible for unilateral or bilateral CN VI palsy. In men, a common primary site of cancer metastasis to the clivus is the prostate. Eye doctors must look closely for evidence of even subtle abduction deficits in all patients with a history of prostate cancer. Early detection can lead to improved medical treatment and extended life expectancy.

Arachnoid Cyst Resulting in Sixth Nerve Palsy in a Child

A 2 year old presented with incomitant esotropia and abduction deficit consistent with sixth nerve palsy. Neuroimaging revealed an arachnoid cyst on the left. Neurosurgical shunting followed by strabismus surgery relieved the abduction deficit and esotropia. An arachnoid cyst may be a rare cause of acquired sixth nerve palsy and strabismus in children.

Acute Comitant Esotropia

&lt;h4&gt;EXCERPT&lt;/h4&gt; &lt;p&gt;&lt;b&gt;Wagner&lt;/b&gt;: A 4-year-old boy had an acute onset of a right esotropia and double vision for 1 day. He had good ophthalmologic care previously for a right nasolacrimal duct obstruction that required intubation approximately 8 months earlier. I don’t think this is related, but 2 weeks prior to presentation he arrived at the emergency department with a silicone tube out on his cheek, which was then removed.&lt;/p&gt; &lt;p&gt;At presentation, he did not appear distressed, although he closed his right eye frequently. He was afebrile. He had no history of recent viral illnesses. Visual acuity was 20/30 in each eye. There was no afferent pupillary defect. He had a right esotropia of 25 PD at distance and near that seemed to be comitant. Nothing else could be seen in full ocular motility. No signs of an abduction deficit were seen in either eye. Cycloplegic retinoscopy results were +2.50 in each eye. The dilated retinal examination revealed blurred optic disk margins in both eyes. I wouldn’t say it was frank papilledema, but it was abnormal. Results of his recent eye examination 2 or 3 months prior were normal.&lt;/p&gt; &lt;p&gt;This 4-year-old boy, who looks good and plays well, is closing his right eye with measurable strabismus and the early findings of papilledema. What is a working diagnosis, and what would be your next step?&lt;/p&gt; &lt;p&gt;&lt;b&gt;Lueder&lt;/b&gt;: I would presume that he had increased intracranial pressure. The results of his previous examination were normal. His vision was normal, and now he is presenting with strabismus. This is not a child with an acute esotropia who is otherwise fine neurologically. I’d be looking at something intracranial and trying to determine if a mass is present.&lt;/p&gt;

Isolated Sixth Cranial Nerve Palsy in Preeclampsia

Isolated Sixth Cranial Nerve Palsy in Preeclampsia

Comparison of Orbital Magnetic Resonance Imaging in Duane Syndrome and Abducens Palsy

To help resolve the clinical ambiguity between Duane syndrome with severe abduction deficit and abducens palsy, we performed orbital magnetic resonance imaging (MRI) to qualify abnormalities of the lateral rectus (LR) muscle in these entities. Prospective observational case series. Orbital MRI was performed in 13 subjects with Duane syndrome (19 eyes), 10 subjects with chronic abducens palsy (10 eyes), and 10 orthotropic control subjects (18 eyes). High-resolution, surface coil, T(1)-weighted MRI was used to obtain contiguous, 2-mm thick quasi-coronal images of the orbits in central gaze. Digital image analysis was used to quantify cross-sectional area of the ipsilesional and contralesional LR to provide comparison with control measurements. Mean maximum LR cross-sectional area in Duane syndrome was statistically similar to control (P = .454) and contralesional LR cross-sectional area (P = .227). However, in chronic abducens palsy, mean maximum ipsilesional LR cross-sectional area was markedly smaller than contralesional (P = .003) and control cross-sectional areas (P < .0001), as well as smaller than the LR in Duane syndrome (P= .0017). The LR muscle in abducens palsy exhibits profound atrophy. The sparing of the LR in Duane syndrome from denervation atrophy despite absence of normal abducens innervation suggests existence of alternative LR innervation. High-resolution MRI can noninvasively demonstrate LR muscle size and distinguish Duane syndrome from chronic abducens palsy in uncertain cases.

Horner Syndrome and Ipsilateral Abduction Deficit Attributed to Giant Cell Arteritis

We recently examined a 77-year-old woman with Horner syndrome and ipsilateral abduction deficit that we attribute to giant cell arteritis (GCA). This combination of findings has not been previously reported in GCA. She had a history of hypertension, hyperlipidemia, atrial fibrillation, congestive heart failure, and colon carcinoma. Six weeks before presentation, she developed bitemporal headaches. She had stopped eating solids as a result of jaw claudication and had lost weight. She had diffuse myalgia and was unable to sleep well because of bilateral scalp tenderness. She noticed binocular horizontal double vision that was worse on left gaze and had experienced numerous episodes of blurring of vision in both eyes. Her family noted drooping of her left upper lid. Neuro-ophthalmic examination revealed a best-corrected visual acuity of 20/40 in both eyes consistent with cataracts. Visual fields and color vision were normal. Left upper lid ptosis of 2 mm was present. In dim light, the right pupil measured 5.5 mm and the left 3.5 mm. In bright light, the right pupil measured 4.5 mm and the left 3 mm. Both pupils reacted briskly to light without afferent defect. Horner syndrome was confirmed by instilling 10% cocaine into both eyes (Fig. 1). (Hydroxyamphetamine was not instilled as a result of its unavailability.) Ocular ductions were full except for reduced abduction of the left eye. There was a 12 prism-diopter esotropia in primary position increasing to 20 on left gaze. The fundus examination was normal.FIG. 1: Left Horner syndrome. After instillation of one drop of a 10% cocaine solution, the right pupil has become dilated and the left pupil has remained small. Left upper lid ptosis is evident.Temporal arterial pulsation was absent and the scalp was tender, but there was no thickening or nodularity. Westergren erythrocyte sedimentation rate (ESR) was 57, C-reactive protein was 4.1, and platelet count was 475,000. MRI of the brain with contrast was normal and magnetic resonance angiography showed an incidental small basilar tip aneurysm. We made a diagnosis of GCA with concurrent Horner syndrome and left lateral rectus paresis. Intravenous methylprednisolone at a dosage of 250 mg per day and 80 mg oral prednisone per day was initiated. She was also started on alendronate, calcium with vitamin D, and ranitidine. Temporal artery biopsy on the left side was consistent with giant cell arteritis (Fig. 2).FIG. 2: Temporal artery biopsy shows inflammatory cells in the vessel wall. Inset shows giant cells (arrows).Within a week of initiation of treatment, she had resolution of diplopia, headaches, and temporal scalp pain. Ptosis and pupillary abnormality remained unchanged. ESR, C-reactive protein, and platelet counts normalized in a week. Prednisone was tapered slowly over 18 months. Horner syndrome resulting from GCA is very rare. Three cases of GCA and Horner syndrome have been reported in the English literature (1-3). Bell et al (1) reported a patient with GCA and Horner syndrome resulting from a brainstem stroke. Askari et al (2) reported a patient with internuclear ophthalmoplegia and Horner syndrome resulting from presumed giant cell arteritis. Bromfield and Slakter (3) reported a patient with GCA with postganglionic Horner syndrome. To our knowledge, no one has reported the combination of Horner syndrome and ipsilateral abduction deficit in GCA. We were unable to perform the hydroxyamphetamine test to confirm the location of the lesion. The absence of brainstem and spinal cord signs and a normal brain MRI argue against a preganglionic location. Hollenhorst et al (4) suggested that paresis of different extraocular muscles at different times is the result of ischemia of the muscles or the nerve and not a brainstem lesion. Barricks et al (5) found extraocular muscle ischemia at autopsy in a patient with bilateral ophthalmoplegia resulting from GCA. Sibony and Lessell (6) reported aberrant regeneration in a patient with pupil-sparing third cranial nerve palsy resulting from GCA and suggested that ophthalmoplegia is neurogenic rather than myogenic. Meadows (7) suggested ischemia of vasa nervorum, whereas Martin (8) suggested ischemia of the extraocular muscles as the likely cause of diplopia in GCA. In our patient, the combination of Horner syndrome and an ipsilateral abduction deficit suggests a cavernous sinus lesion. Within the cavernous sinus, the postganglionic sympathetic fibers travel with the sixth cranial nerve lateral to the internal carotid artery. Bromfield and Slakter (3) proposed that granulomatous inflammation of the internal carotid artery might directly involve the sympathetic fibers. We believe that the sixth cranial nerve in our patient could have been similarly affected. Guruswami Arunagiri, MD Shanmugam Santhi, MD Thomas Harrington, MD Departments of Ophthalmology and Rheumatology, Geisinger Medical Center, Danville, PA

Saccade dynamics in peripheral vs central sixth nerve palsies

To investigate differences between peripheral idiopathic and central sixth nerve palsies from brainstem damage by comparing peak velocities and durations of horizontal saccades. Fourteen patients with unilateral incomplete sixth nerve palsies caused by idiopathic, presumed ischemic, peripheral damage, 5 with incomplete central (fascicular) palsy caused by brainstem lesions, and 10 controls were studied. Palsies under 1 month in duration were designated as acute and those of longer duration were chronic. Among peripheral palsies, five were acute, nine were chronic. Among central palsies, two were acute, three were chronic. Subjects made +/- 10 deg horizontal saccades while wearing search coils. Serial recordings were made in seven patients with acute palsy (five peripheral, two central). Centrifugal abducting saccadic velocities in the paretic eye were subnormal in both central and peripheral acute palsies, as anticipated from lateral rectus weakness. In chronic central palsies, abducting velocities in the paretic eye remained reduced. However, in chronic peripheral palsies, velocities became normal in the tested range of excursion, within 2 months of onset, despite persisting abduction deficit. Saccade peak velocities are reduced and their durations are prolonged in the field of action of acutely palsied peripheral and central nerves. Speeds remain reduced in chronic central (fascicular) palsies, consistent with limited regeneration within the brain. Saccade speeds are repaired in chronic peripheral palsies, probably by remyelination and axonal regeneration, and perhaps also by central monocular adaptation of innervation selectively to the paretic eye, in order to drive both eyes rapidly and simultaneously into the paretic field of motion.

Transient downbeat nystagmus from West Nile virus encephalomyelitis

Most human West Nile virus (WNV) infections are asymptomatic, but a minority of patients develops neuroinvasive disease, including a poliomyelitis-like syndrome.1 Reported ophthalmologic manifestations include optic neuritis, uveitis, multifocal choroiditis, ocular motor palsies, and opsoclonus.2–4 We cared for a patient with WNV who developed downbeat nystagmus and ocular flutter with acute areflexic quadriparesis. A 64-year-old previously healthy retired steel mill worker presented after 7 days of leg and arm pain and 4 days of progressive weakness. He reported horizontal binocular diplopia, decreased strength, mild dyspnea, and arm tremor. On admission, temperature was 39.2 °C. He was oriented and followed requests. Visual fields were full. Pupils were equal and reactive. He had a mild esotropia in the primary position, with a partial right abduction deficit with right lateral gaze. Downbeat nystagmus was present in primary gaze, and the amplitude increased in up gaze and lateral gaze (video, on the Neurology Web site at www.neurology.org). Pursuit was saccadic in all directions, but particularly in vertical gaze. He had intermittent …

HOXA1 mutations are not a common cause of Duane anomaly

HOXA1 mutations are not a common cause of Duane anomaly

Chronic Lymphocytic Meningitis in an Adolescent

A 13-year-old white female complained of a 2-month history of diffuse, often intense headaches and a 1-month history of intermittent nausea, vomiting, and anorexia with intermittent low-grade fevers. An outpatient evaluation included 2 negative rapid screens for group A streptococcus, a negative heterophil antibody test, a negative rapid influenza antigen test, and a normal gall bladder ultrasound. Symptoms persisted despite a 5-day course of azithromycin. She was hospitalized secondary to worsening headaches, nausea, and the onset of diplopia. The patient lived in a rural area in Kentucky and had multiple animal exposures. Family pets included a dog and a fish, and the patient’s home was infested with mice. Just before the onset of her symptoms, the patient had explored a deserted house and found a dead opossum, but she denied touching it. She had recently visited a local stockyard and denied direct contact with the cattle, but did have contact with cow manure; her face had been splashed with manure while she was riding an all-terrain vehicle across a cow pasture. On initial evaluation, the patient had a temperature of 36.4� C, a heart rate of 80 bpm, and blood pressure of116/57 mm Hg. Her neurologic exam was remarkable for bilateral papilledema, left esotropia, left abduction deficit, mild left upper lid ptosis, and mild gait ataxia. Respiratory, cardiovascular, joint, extremity, and lymph node examinations were normal. At admission, the white blood cell (WBC) count was 7.7 3 10 3 /mL with 59% neutrophils, 29% lymphocytes, and 9% monocytes. Hemoglobin was 13.8 gm/dL, and platelets were 371 3 10 3 /mL. Electrolytes and serum transaminases, bilirubin, and albumin were within normal limits. Antibodies to human immunodeficiency virus I and II and Borrelia burgdorferi were negative. A serum rapid plasma reagin (RPR) for syphilis was also negative. Blood and urine cultures were sterile. A tuberculin skin test was negative with positive response to a Candida control. Cerebrospinal fluid (CSF) was clear and colorless with an opening pressure of 310 mm Hg. There were 440 WBCs/mL (7% neutrophils, 80% lymphocytes, 13% monocytes) and 130 red blood cells (RBCs)/mL. CSF protein was 79 mg/dL, and glucose was 38 mg/dL. Negative CSF studies included bacterial and mycobacterial cultures, acid-fast bacilli stain, VDRL, adenovirus polymerase chain reaction, and cryptococcal antigen. Magnetic resonance imaging (MRI) of the brain with and without gadolinium at the time of admission and 5 days later demonstrated no meningeal enhancement or focal lesions. Electroencephalography and echocardiography were also normal. A chest radiograph revealed no parenchymal disease or lymphadenopathy. Cefotaxime was begun empirically, but was discontinued after 4 days. An evaluation for immunodeficiency included normal quantitative immunoglobulins (IgG, 1100 mg/dL; IgM, 172 mg/dL; IgA, not done). Peripheral blood immunotyping revealed normal percentages and absolute numbers of T and B cells with normal lymphocyte response to phytohemagglutinin stimulation.

Ocular Complications of Sigmoid Sinus Thrombosis From L-asparaginase

This article presents a 9-year-old boy with acute lymphocytic leukemia who developed ocular complications after beginning treatment with L-asparaginase. Ocular motor examination revealed esotropia with a right abduction deficit, consistent with a sixth nerve palsy, and funduscopy revealed bilateral optic disc edema. Thrombosis of the right sigmoid sinus was noted on magnetic resonance imaging and magnetic resonance venography. Symptoms gradually resolved after discontinuing L-asparaginase and treatment with heparin.

Unilateral Involuntary Eyelid Closure Induced by Diplopia That Did Not Remit With Contact Lens Occlusion

We report an unusual case of unilateral blepharospasm secondary to diplopia that did not remit even with contact lens occlusion. A 50-year-old woman had diplopia on right gaze for 3 years. Examination revealed a right face turn, a complete right abduction deficit, and a partial deficit of right adduction and supraduction. The eyelids and pupils were normal. Magnetic resonance imaging revealed a right intracavernous meningioma. During follow-up, diminished supraduction of the OD and synkinetic elevation of the lid on adduction and infraduction developed, as well as absence of right incyclotorsion on attempted downward gaze, attributed to aberrant regeneration of a palsied third cranial nerve (Fig. 1). The face-turn increased and involuntary unilateral closure of the OD lids developed that was attributed to a desire to eliminate diplopia (Fig. 2).FIG. 1: Right third cranial nerve palsy. The patient's lids have been elevated and she has been asked to direct her gaze up, down, left, and right (see arrows). There is limitation principally of supraduction and abduction of the OD.FIG. 2: Involuntary blepharospasm, right. The left panel shows the patient at rest with involuntary closure of the OD lids. This occurred when she was awake and also persisted when the OD was occluded with a contact lens. The right panel shows that she could, with effort, open her OD for short periods. There is moderate ptosis caused by partial weakness of the levator palpebrae superioris.Botulinum toxin injected into the right medial rectus failed to relieve primary position diplopia, so a right occlusive contact lens was fitted. Despite relief of the diplopia, the involuntary OD lid closure persisted. When the occlusive contact lens was removed and she occluded her OS, the OD lids opened briefly, but she experienced vertigo. A right upper lid crutch in conjunction with the right occlusive lens was unsuccessful. She was finally treated with botulinum toxin injection into the right orbicularis muscle. With the occluding contact lens in place in the OD, the OD lids remained open. Several triggers of blepharospasm have been reported. These include bright lights, air, and cold (1). Elston (2) found that 57% of 272 blepharospasm patients had ophthalmological symptoms at the onset, mostly irritation and photophobia, but there was no evidence of ocular disease on examination. Jankovic and Orman (3) reported the lack of associated ocular signs in patients with blepharospasm who described ocular symptoms. Ocular irritation as a result of lagophthalmos from Bell's palsy is thought also to be a precipitating factor in those predisposed to blepharospasm (4). Precipitating triggers have been noted in other dystonias, such as local hand injury in those with writer's cramp (2). It is possible that diplopia-activated brain stem mechanisms subserving unilateral eye closure in our patient resulted in involuntary eye closure that persisted despite contact lens occlusion and eventually required botulinum toxin treatment. Eugene Tay MSc, MRCSEd, MRCOphth Gordon T. Plant MA, MD, FRCP Moorfields Eye Hospital and Royal National Hospital for Neurology and Neurosurgery; London, England; E-mail: [email protected]

Outbreak of Life-Threatening Thiamine Deficiency in Infants in Israel Caused by a Defective Soy-Based Formula

Objective. Between October and No- vember 2003, several infants with encephalopathy were hospitalized in pediatric intensive care units in Israel. Two died of cardiomyopathy. Analysis of the accumu- lated data showed that all had been fed the same brand of soy-based formula (Remedia Super Soya 1), specifi- cally manufactured for the Israeli market. The source was identified on November 6, 2003, when a 5.5-month-old infant was admitted to Sourasky Medical Center with upbeat nystagmus, ophthalmoplegia, and vomiting. Wer- nicke's encephalopathy was suspected, and treatment with supplementary thiamine was started. His condition improved within hours. Detailed history revealed that the infant was being fed the same formula, raising sus- picions that it was deficient in thiamine. The formula was tested by the Israeli public health authorities, and the thiamine level was found to be undetectable ( 25% indicates severe deficiency. Blood lactate levels (normal: 0.5-2 mmol/L) were measured in 6 infants, cerebrospinal fluid lactate in 2 (normal: 0.5-2 mmol/L), and blood pyruvate in 4 (nor- mal: 0.03-0.08 mmol/L). The diagnostic criteria for thia- mine deficiency were abnormal transketolase activity and/or unexplained lactic acidosis. Treatment consisted of intramuscular thiamine 50 mg/day for 14 days com- bined with a switch to another infant formula. Results. Early symptoms were nonspecific and in- cluded mainly vomiting (n 8), lethargy (n 7), irrita- bility (n 5), abdominal distension (n 4), diarrhea (n 4), respiratory symptoms (n 4), developmental delay (n 3), and failure to thrive (n 2). Infection was found in all cases. Six infants were admitted with fever. One pa- tient had clinical dysentery and group C Salmonella sep- sis; the others had mild infection: acute gastroenteritis (n 2); upper respiratory infection (n 2); and broncho- pneumonia, acute bronchitis, and viral infection (n 1 each). Two infants were treated with antibiotics. Three infants had neurologic symptoms of ophthalmoplegia with bilateral abduction deficit with or without upbeat nystagmus. All 3 had blood lactic acidosis, and 2 had high cerebrospinal fluid lactate levels. Patient 1, our in- dex case, was hospitalized for upbeat nystagmus and ophthalmoplegia, in addition to daily vomiting episodes since 4 months of age and weight loss of 0.5 kg. Findings on brain computed tomography were normal. Blood lac- tate levels were high, and TPPE was 37.8%. Brain mag- netic resonance imaging (MRI) revealed no abnormali- ties. Patient 2, who presented at 5 months with lethargy, vomiting, grunting, and abdominal tenderness, was found to have intussusception on abdominal ultrasound and underwent 2 attempts at reduction with air enema several hours apart. However, the lethargy failed to re- solve and ophthalmoplegia appeared the next day, lead- ing to suspicions of Wernicke's encephalopathy. Labora- tory tests showed severe thiamine deficiency (TPPE 31.2%). In patients 1 and 2, treatment led to complete resolution of symptoms. The third infant, a 5-month-old girl, was admitted on October 10, 2003, well before the outbreak was recognized, with vomiting, fever, and oph- thalmoplegia. Her condition deteriorated to seizures, ap- nea, and coma. Brain MRI showed a bilateral symmetri- cal hyperintense signal in the basal ganglia, mamillary bodies, and periaqueductal gray matter. Suspecting a metabolic disease, vitamins were added to the intrave- nous solution, including thiamine 250 mg twice a day. Clinical improvement was noted 1 day later. TPPE assay performed after treatment with thiamine was started was still abnormal (17.6%). Her formula was substituted after 4 weeks, after the announcement about the thiamine deficiency. Although the MRI findings improved 5 weeks later, the infant had sequelae of ophthalmoplegia