Bilateral Cranial Nerve Palsy Research Articles

Bilateral ptosis without upward gaze palsy: Unusual presentation of midbrain tuberculoma

Central nervous system tuberculoma can have variable presentations depending on the site and number of tuberculomas. We are reporting a rare case of an 11-year-old male child presenting with ptosis and ataxia. Clinical examination revealed bilateral partial 3rd cranial nerve palsy (ptosis without any upward gaze palsy) associated with dysdiadochokinesia and ataxia on the right side. Magnetic resonance imaging of the brain revealed a single ring-enhancing lesion in the dorsal midbrain with perifocal edema. Magnetic resonance spectroscopy provided the etiological information as tuberculoma.

Ischemic stroke presenting with bilateral 6th cranial nerve palsy, nausea and vomiting

Ischemic stroke presenting with bilateral 6th cranial nerve palsy, nausea and vomiting

Bilateral Cranial Nerve Palsy as a Harbinger of Acute Lymphoblastic Leukemia

Bilateral Cranial Nerve Palsy as a Harbinger of Acute Lymphoblastic Leukemia

E-031 cervical access and use of peripheral vascular stent for venous sinus stenosis: technical considerations and mid-term angiographic and clinical results

Introduction Venous sinus stenting has become increasingly accepted as an acceptable treatment for idiopathic intracranial hypertension (IIH) in selected patients who fail medical treatment and in whom a hemodynamically significant stenosis and pressure gradient can be measured. Technical challenges may include navigating past the jugular bulb or area of stenosis without prolapsing the supporting catheter into the heart and the fact that all carotid stenting systems use a 0.014” wire, which often does not provide adequate support in tortuous venous anatomy. Methods We review our initial experience using a peripheral vascular stent using brachial or jugular access, which is delivered over a 0.035 wire. In addition, we review the clinical features and angiographic outcomes of these patients. Results Three patients were available with at least 6 month angiographic follow-up. One patient presented with typical IIH symptoms, one with failure of medical and endovascular treatment for sinus thrombosis, and one with acute severe vision loss, intracranial hypertension and sinus stenosis without obvious thrombus. All patients had documented papilledema and the two acute patients also had bilateral 6th cranial nerve palsies. All patients had venous pressure gradients >20 mmHg across the stenotic segment. All stents were deployed across the target segment from either brachial or cervical venous access with a 6F short sheath and a 0.035 Rosen or glide wire. All stents were 10mm diameter, flexible, open cell stents (LifeStar: Bard PV: Tempe, AZ). One patient required two stents and the remainder required only one. Delayed imaging showed patency of all stents without stenosis (range 6–15 months.) Anti-platelet medications were used in all patients_one with aspirin only, two with aspirin and clopidogrel. All patients had improvement of clinical and ophthalmological symptoms. Conclusions Venous sinus stenting is a promising treatment for restoration of sinus patency and improvement of clinical symptoms in selected patients. Cervical access and the support of a 0.035” wire makes delivery of the device simpler compared to use of standard carotid stents. Disclosures A. Carlson: None. A. Delu: None. C. Taylor: None.

Strabismus and diplopia in a patient with acute myeloid leukemia.

Patient: Male, 64Final Diagnosis: Acute myeloid leukemia (AML)Symptoms: —Medication: —Clinical Procedure: —Specialty: —Objective:Unusual clinical courseBackground:Central nervous system (CNS) involvement is a sporadic presenting finding in patients with acute myeloid leukemia (AML) both at diagnosis and at relapse. Moreover patients with CNS localization are often asymptomatic, while sometimes show meningeal signs and symptoms or, extremely rarely, signs of cranial nerve impairment.Case Report:Here we report on a patient with refractory AML who suddenly developed strabismus and diplopia. Both neurological and ophtalmologic examinations were suggestive of a bilateral VI cranial nerve palsy. Noteworthy, both a cranial CT and MRI were substantially normal, while a rachicentesis was performed and cerebrospinal fluid examination was clearly suggestive of a meningeal involvement by AML.Conclusions:This is to our knowledge the first reported case in which the clinical picture of meningeal localization in an AML patient was dominated by an isolated abducens cranial nerve impairment. Moreover it highlights as unexplained strabismus and diplopia can be considered as a potential sign of CNS involvement, even if conventional imaging is negative.

Central retinal vein occlusion after gamma knife radiosurgery for cavernous sinus dural arteriovenous fistula

A 70-year-old man presented to our clinic complaining of binocular diplopia and persistent redness in the left eye for 5 months. Conjunctival vessels were dilated and tortuous in both eyes. Intraocular pressure was 16 mmHg in the right eye and 23 mmHg in the left eye. Complete bilateral sixth cranial nerve and partial left third cranial nerve palsies were noted. Brain computed tomography and cerebral angiography revealed a Barrow Type C dural arteriovenous fistula. The patient underwent gamma knife radiosurgery (GKRS) at a dose of 18 Gy and a target volume of 3.3 mL. Within 1 month of GKRS, the patient reported an improvement in diplopia, but 4 months following the procedure, he experienced micropsia, metamorphopsia, and darkness in his left eye. Prominent flame-shaped hemorrhages and retinal edema, the typical picture of central retinal vein occlusion (CRVO), developed in his left eye. Intraocular pressure in this eye was controlled with antiglaucoma topical medication. 5 months later, the left retina had appeared normal. Antiglaucoma drops were gradually tapered and eventually discontinued. The GKRS, used in recent decades, is noninvasive and effective in the treatment of dural arteriovenous fistula. However, CRVO can occur after GKRS. Here, we present, in detail, a clinical course of CRVO that developed after GKRS. Our findings may be useful for further understanding of this rare complication.

Idiopathic Intracranial Hypertension in Sickle Cell Disease: A Paediatric Case Report

In this article, we present the case of a 12-year-old girl with sickle cell disease (SCD), who presented with the severe headache. She had bilateral 6th cranial nerve palsy and papilloedema. The common sickle cell-related vascular causes of headache were ruled out by neuro-imaging. She then had a lumbar puncture and was diagnosed with idiopathic intracranial hypertension (IIH). This case demonstrates that IIH can affect younger children with SCD and should form a part of differential diagnosis when investigating causes of headache in SCD.

Scleral contact lens management of bilateral exposure and neurotrophic keratopathy

We report an interesting case of therapeutic scleral lens management of bilateral exposure and neurotrophic keratopathy resulting from bilateral cranial nerve (CN) palsies including V, VI and VII, which caused lagophthalmos and anaesthetic corneas. Subsequent development of severe exposure keratitis with vascularisation and keratinisation of the inferior cornea was previously treated with intensive ocular lubrication, botulinum toxin injections to the upper eyelid levator muscle, temporary tarsorrhophies, bilateral amniotic membrane grafts, punctal plugs, lid taping, gold eyelid weights and soft bandage contact lenses. Corneal integrity was re-established but visual acuity remained significantly compromised by corneal vascularisation, scarring and keratin deposits. Visions on presentation to the contact lens department were R 1.90 logMAR, L 1.86 logMAR. Therapeutic, high Dk, non-fenestrated, saline filled, scleral lenses were fitted. Daily wear of these lenses have protected and hydrated the cornea, enabling corneal surface recovery whilst retaining visual and social function. The visual acuities 6 months post-scleral fitting with lenses in situ are R 0.90 logMAR and L logMAR 0.70.

A Case of Bilateral Lower Cranial Nerve Palsies After Base of Skull Trauma With Complex Management Issues

Fractures of the skull base can cause lower cranial nerve palsies because of involvement of the nerves as they traverse the skull. A variety of syndromes have been described, often involving multiple nerves. These are most commonly unilateral, and only a handful of cases of bilateral cranial nerve involvement have been reported. We describe a 64-year-old man with occipital condylar fracture complicated by bilateral palsies of IX and X nerves associated with dramatic physiological derangement causing severe management challenges. Apart from debilitating postural hypotension, he developed dysphagia, severe gastrointestinal dysmotility, issues with airway protection as well as airway obstruction, increased oropharyngeal secretions and variable respiratory control. This is the first report of a patient with traumatic bilateral cranial nerve IX and X nerve palsies. This detailed report and the summary of all 6 previous case reports of traumatic bilateral lower cranial nerve palsies illustrate clinical features, treatment strategies, and outcomes of these rare events.

Presentation of Bilateral Peripheral Seventh Cranial Nerve Palsy in an HIV Patient

Neurological manifestations in patients infected with human immunodeficiency virus can significantly increase overall morbidity and mortality. These complications are neither limited to a specific location in the nervous system nor a focal time period in the disease's progression. A literature review yielded several cases of peripheral facial palsy associated with HIV seropositivity, but few cases have been reported where the patient had bilateral peripheral facial palsy. In this paper, we present a patient with bilateral peripheral facial palsy and aseptic meningitis in the context of newly diagnosed HIV.

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Sir:FigureThe timing of facial reanimation surgery in congenital facial palsy syndrome has been recommended in the child at approximately 5 years of age for psychosocial rehabilitation.1 However, the dental occlusion and facial proportion actually are not yet mature, and the effects of muscle transfer on a developing facial skeleton are difficult to predict. There is a lack of long-term follow-up for those children with Möbius syndrome who underwent reanimation surgery reported in the literature. The series of Möbius and Möbius-like syndrome patients in the article by Bianchi et al.2 had rather minor severity. The absence of muscle activity in the upper lip causes excessive forward growth of the maxilla, whereas downward growth is arrested. For cases in which dental problems are corrected in the Möbius patient, we strongly recommend that orthognathic surgery precede microsurgery, especially in the patient lacking normal masticatory muscle function and with possible developmentally abnormal facial proportions. For poor dental occlusion and a longer middle and lower face, orthognathic surgery is worthwhile before “smile” reconstruction. The bony work, mainly addressed during orthognathic surgery, should be scheduled in late adolescence. Surgically, the entire maxilla can be moved forward up to 10 mm with good stability and as far as 10 to 15 mm with excellent stability. The procedure is technically exacting but not otherwise problematic.3 Orthognathic surgery should be performed first to obtain a class I dental relationship and to optimize static facial aesthetics and substantially reduce drooling, oral incompetence, and speech difficulties.4 Microsurgical functional muscle transfers may be delayed until bone maturation and reshaping by orthognathic surgery. Facial animation and nonhollowed cheeks are later achieved in a second-stage procedure. The severity of cranial nerve involvement in Möbius syndrome is variable. The classic technique of hypoglossal nerve transfer in facial nerve reconstruction with the resultant hemiglossal paralysis may lead to significant morbidity, including speech, mastication, and swallowing difficulties. These problems may be further compounded if the patient has bilateral or multiple lower cranial nerve palsies (e.g., Möbius syndrome). A weaker facial response and longer recovery time have been observed even incising the hypoglossal nerve distal to the descendens hypoglossi branch to avoid compromising adequate tongue motor innervation.5 The masseter motor branch of the fifth cranial nerve is another tempting source, because free muscle transfer can be connected directly without the need for nerve grafting. The masseter muscle has to be stripped during orthognathic surgery, and that may add to the degree of difficulty in nerve end identification later. For these reasons—risk of aspiration, speech and swallowing difficulties, and the need for bilateral motor nerves—we selected the spinal accessory nerve instead of other motor branches in contemplating functioning muscle nerve repair for patients with Möbius syndrome. By far the most often selected nerve source in the series review was the spinal accessory nerve, which is the least involved nerve reported in the literature.6 Because there are various cranial nerves involved in Möbius syndrome, no single standard procedure for dynamic restoration should be overemphasized. Sophia Chia-Ning Chang, M.D., Ph.D. Department of Plastic Surgery, China Medical University Hospital, and, School of Medicine, China Medical University, Taichung, Taiwan David Chwei-Chin Chuang, M.D. Department of Plastic Surgery, Chang Gung Memorial Hospital, Chang Gung University College of Medicine, Taoyuan, Taiwan DISCLOSURE Neither of the authors has any commercial associations or financial disclosures.

Rituximab responsive multiple radiculopathies and cranial nerve palsies in association with chronic lymphocytic leukaemia

We describe a previously healthy 53 year old woman with a progressive multifocal neurological syndrome. She developed gradually progressive patchy numbness and horizontal diplopia over 3 years, night sweats and 15 kg weight loss over 1 year, and weakness of the face and four limbs over 9 months. Previous treatment for her symptoms included dual anti-platelet agents then warfarin for a positive lupus anticoagulant, oral fludarabine (50 mg for 5 days) following a diagnosis of mild chronic lymphocytic leukaemia (CLL) on bone marrow biopsy, and two courses of oral steroids (25 mg/day prednisolone; 20 mg/day dexamethosone each tapered over 4 weeks). Neurological symptoms progressed despite these treatments. On examination she mobilised with a frame. There were partial right III, VI and bilateral VII cranial nerve palsies. There was wasting of intrinsic hand muscles, distal lower limb muscles and right deltoid. There was asymmetric proximal and distal weakness in all limbs ranging from no active contraction of right deltoid, flicker of contraction of left hamstrings to mild weakness of ankle dorsiflexors. There was patchy loss of reflexes and flexor plantars. She had sensory loss to light touch and pinprick in multiple dermatomes. The working diagnosis was CLL-associated immunemediated radiculopathies and cranial neuropathies. She was treated with rituximab 375 mg/m and hydrocortisone 100 mg weekly for 8 weeks without additional steroids (repeated previous steroid-associated central serous retinopathy). She made a near complete response. Three months after the rituximab her only residual symptom was with feeling a little unsteady. The only abnormalities on neurological examination were slight diplopia on right gaze and reduced vibration sensation to the ankles. Investigations (Table 1) had dramatically improved. She was maintained on oral cyclophosphamide 50 mg/day, however, had a minor relapse after 5 months and received a repeat rituximab course with good effect. She is neurologically stable on maintenance rituximab 375 mg/m every 3 months. Mechanisms by which CLL could be associated with neurological symptoms include direct leukaemic infiltration, indirectly through immune-mediated effects, opportunistic infection related, treatment related or chance association. Neurological syndromes are found in 11% of CLL patients mostly relating to opportunistic infections [1]. Cases of CLL associated with GBS [2, 3], MillerFisher syndrome [4], subacute IDP [5], mononeuritis multiplex [5] and polyneuropathy [5] are reported. A case with multiple radiculopathies and cranial neuropathies was reported recently [6], however, direct leukemic infiltration of nerve roots was evident on imaging and CSF. In our patient repeated MRI and CSF examinations showed no evidence of direct leukaemic infiltration. J. M. Morrow (&) M. P. Lunn M. M. Reilly MRC Centre for Neuromuscular Diseases, Department of Molecular Neurosciences, UCL Institute of Neurology, National Hospital for Neurology and Neurosurgery, Box 102, WC1N 3BG London, UK e-mail: j.morrow@ion.ucl.ac.uk

Murine typhus returns to New South Wales: a case of isolated meningoencephalitis with raised intracranial pressure

Murine typhus (MT) occurs worldwide, but, in Australia, is only regularly diagnosed in south-west Western Australia. Meningoencephalitis is an uncommon complication of MT, often accompanied by rash or systemic involvement. We report a case of MT presenting exclusively with meningoencephalitis, raised intracranial pressure, papilloedema and bilateral 6th cranial nerve palsies. MT should be considered in patients with "aseptic" meningitis or meningoencephalitis, even in the absence of other typical features of a typhus-like illness.

Acute Spinal Epidural Hematoma and Cranial Interdural Hematoma Due to a Rupture of a Posterior Communicating Artery Aneurysm: Case Report

We describe an unusual presentation of a ruptured aneurysm of the posterior communicating artery with an acute intracranial hematoma between the dural layers associated with an acute spinal epidural hematoma descending to L1. A 35-year-old woman presented 3 hours after ictus with a postcoital headache, neck stiffness, and bilateral abducens cranial nerve palsy. No other neurological deficits were present. Clinically, she had a subarachnoid hemorrhage World Federation of Neurosurgical Societies grade 1. CT scan demonstrates an acute subdural hematoma, extending from the right parasellar region, around the clivus, tentorium, and falx. Angio-CT showed a posterior communicating artery aneurysm and an anterior communicating artery aneurysm and an extension of the hematoma to the cervical spine. This justified a spinal and cerebral MRI that confirmed an extension of the hematoma to the epidural space at the cervical, thoracic, and lumbar levels. Three-dimensional digital subtraction angiography confirmed aneurysms on the right posterior communicating artery and on the anterior communicating artery. Both aneurysms were completely occluded by coiling. With reference to the concept of the cranial subdural compartment described in studies conducted using an electron microscope, this group of hematomas was classified as interdural. Ruptured aneurysm of the posterior communicating artery may cause cranial acute interdural hematoma with a typical subarachnoid hemorrhage clinical presentation, and it rarely can extend to spinal epidural space.

Bilateral lower motor neuron facial nerve palsy due to HIV seroconversion

Case Study

Progressive Multiple Bilateral Cranial Nerve Palsies Due to Malignant Peripheral Nerve Sheath Tumour

Malignant peripheral nerve sheath tumours (MPNST) are rare tumours that usually do not involve cranial nerves. The case we report is to the best of our knowledge the first description of bilateral progressive cranial nerve palsies due to a MPNST in a patient without evidence of Neurofibromatosis.

Ocular Motor and Imaging Abnormalities of Midbrain Dysfunction in Osmotic Demyelination Syndrome

After rapid correction of severe hyponatremia, a 36-year-old man developed osmotic demyelination syndrome (ODS), manifested neurologically by impaired cognition, extremity weakness, bilateral third cranial nerve palsies, and gaze-evoked upbeat and rotary nystagmus. Brain MRI showed restricted diffusion in the rostral midbrain and temporal and parietal lobes but not in the pons. Over several weeks, all neurologic and imaging deficits resolved. This is the first report to document ocular motor abnormalities associated with midbrain dysfunction in ODS.

Primary medulla oblongata germinoma – an unusual posterior fossa tumors in young adults

We encountered 2 patients with germinoma arising from the medulla oblongata in whom preoperative radiological diagnosis was difficult. A 30-year-old woman presented due to aspiration pneumonia caused by bilateral lower cranial nerve palsies, and a 24-year-old man presented with headache caused by obstructive hydrocephalus. In both patients, there was a midline tumor that extended from the lower part of the fourth ventricle to the C1 lamina level. It was well-demarcated and homogeneously enhanced tumor with a slightly high density on plain CT scan. The preoperative diagnosis for both patients was ependymoma. The former patient had persistent lower cranial nerve palsies due to brain stem injury after tumor resection. Both patients achieved complete remission with adjuvant therapy. Fewer than 10 cases of germinoma affecting the medulla oblongata have been reported. Radiological findings resembling those of the pineal region germinoma were observed in the two patients reported here. We would like to stress the importance of remembering germinoma when making a preoperative differential diagnosis of fourth ventricular tumors in young adults.

Spontaneous intracranial hypotension and immediate improvement following epidural blood patch placement demonstrated by intracranial pressure monitoring

The presentation of spontaneous intracranial hypotension (SIH) can be associated with various clinical and neuroimaging features that may impede a rapid diagnosis of this entity. The authors report the case of a patient who presented with bilateral third cranial nerve palsies and bilateral subdural hematomas. Intracranial pressure monitoring proved to be useful in the diagnosis and management of SIH in this patient.

Complete ophthalmoplegia associated with clival fracture following trauma: Case report and clinicoanatomical correlation

Fractures of clivus occur following high energy trauma and are rare. There are a few reports of associated vascular and brainstem injury in literature. We report a patient with transverse clivus fracture and complete ophthalmoplegia and bilateral multiple cranial nerve palsies. A young boy was admitted in altered sensorium following RTA. Initial imaging revealed diffuse pneumocephalus, subarachnoid hemorrhage and diffuse cerebral edema. He was treated conservatively. Clinical review after improvement in sensorium showed that he had visual loss in left eye, bilateral ptosis, complete ophthalmoplegia in horizontal as well as vertical gaze, bilateral trigeminal paresis, right facial weakness and dysarthria. There was right CSF otorrhea. A spiral CT scan revealed transverse clivus fracture extending into bilateral middle cranial base. The fracture line was involving facial canal and mastoid on the right side. An MRI brain demonstrated periaqueductal infarcts and multiple cortical infarcts. He was managed conservatively. In the presence of clinical suspicion of skull base injuries, a spiral CT scan and MRI brain and MR angiography are essential for accurate assessment of bony and neurological involvement. The diagnosis of clivus fracture should prompt the clinician to look for associated brainstem or basilar artery injury.