Idiopathic third and sixth cranial nerve neuritis.

PURPOSE: To present cases with idiopathic third and sixth cranial nerve neuritis. STUDY DESIGN: Retrospective observational study METHODS: The results of high resolution pre- and post- cranial nerve magnetic resonance images (MRI) with three-dimensional sequences for visualizing cranial nerves in patients with third, fourth, and sixth cranial nerve palsies who were treated at the Neuro-ophthalmology Department of Samsung Medical Center were reviewed. Patients with cranial nerve enhancement confirmed by experienced radiologists were identified. The medical records of these patients were reviewed, and their demographics, clinical presentations, laboratory results, and clinical outcomes were analyzed. RESULTS: Of 265 patients with third, fourth, and sixth cranial nerve palsy, 60 were identified by high resolution MRI as having enhancement of the corresponding cranial nerve. Among these, 17 patients with infiltrative, granulomatous, or tumorous lesions were excluded. In addition, 28 patients with identifiable causes of cranial nerve palsy, such as Miller-fisher syndrome, virus infection, or radiation-induced neuropathy, as well as patients with vasculopathic risk factors, were also excluded. Ultimately, a total of 15 patients with idiopathic third and sixth cranial nerve neuritis were included in this study. The mean age of these patients was 43 ± 15 years. Eight patients had sixth cranial nerve palsy, six third cranial nerve palsy (two partial and four complete), and one patient with complete third and sixth cranial nerve palsy. Nine patients received steroid treatment. Eleven patients recovered fully within a period ranging from a few days to one year. Two patients were much improved up to 1 month after initial presentation, but were then ultimately lost to follow-up. Another patient was lost to follow-up after the initial work-up. The other patient lost to follow-up had partially recovered during the first 6 months. CONCLUSIONS: We present patients with idiopathic third and sixth cranial nerve neuritis. They tended to respond well to steroid treatment and to have good prognoses. In order to better understand the long-term prognosis of cranial nerve neuritis and possible association with other neurologic disorders, a larger scale and longer-term study is needed.

Will the real Moebius syndrome please stand up? A systematic review of the literature and statistical cluster analysis of clinical features.

Moebius syndrome is a highly variable syndrome with abducens and facial nerve palsy as core features. Strict diagnostic criteria do not exist and the inconsistency of the associated features makes determination difficult. To determine what features are associated with Moebius syndrome we performed a systematic literature review resulting in a composite case series of 449 individuals labeled with Moebius syndrome. We applied minimum criteria (facial and abducens palsy) to determine the prevalence of associated clinical features in this series. Additionally, we performed statistical cluster analysis to determine which features tended to occur together. Our study comprises the largest series of patients with Moebius syndrome and the first to apply statistical methodology to elucidate clinical relationships. We present evidence for two groups within the Moebius diagnosis. Type 1: exhibiting micrognathia, limb anomalies and feeding/swallowing difficulty that tend to occur together. Type 2: phenotypically diverse but more associated with radiologically detectable neurologic abnormalities and developmental delay.

Electrophysiology of Extraocular Cranial Nerves: Oculomotor, Trochlear, and Abducens Nerve.

The utility of extraocular cranial nerve electrophysiologic recordings lies primarily in the operating room during skull base surgeries. Surgical manipulation during skull base surgeries poses a risk of injury to multiple cranial nerves, including those innervating extraocular muscles. Because tumors distort normal anatomic relationships, it becomes particularly challenging to identify cranial nerve structures. Studies have reported the benefits of using intraoperative spontaneous electromyographic recordings and compound muscle action potentials evoked by electrical stimulation in preventing postoperative neurologic deficits. Apart from surgical applications, electromyography of extraocular muscles has also been used to guide botulinum toxin injections in patients with strabismus and as an adjuvant diagnostic test in myasthenia gravis. In this article, we briefly review the rationale, current available techniques to monitor extraocular cranial nerves, technical difficulties, clinical and surgical applications, as well as future directions for research.

Parálisis bilateral delVI par craneal como manifestación inicial de la meningitis meningocócica / Bilateral abducens nerve palsy as the initial clinical manifestation of meningococcal meningitis

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Factors Associated with Abducens Nerve Recovery in Patients Undergoing Surgical Resection of Sixth Nerve Schwannoma: A Systematic Review and Case Illustration.

BACKGROUND: Limited or no literature exists identifying factors associated with functional nerve recovery in patients undergoing resection of sixth cranial nerve (CN VI) schwannomas. METHODS: A systematic review of literature was performed on CN VI schwannomas that were treated surgically. Synthesizing the findings pooled from the literature, we investigated associations of patient demographics and clinical characteristics with postsurgical CN VI functional recovery in multivariable regression models. In addition, we present the findings of an adolescent woman surgically managed for intracavernous CN VI schwannoma. Complete encasement of the cavernous segment of the internal carotid artery is unique to our case. RESULTS: We synthesized data of 32 patients from 29 studies, and our index case. Overall, the mean age of the patients was 44.0 ± 16.5 years, and approximately 52% (n = 17) were female. Most tumors were left-sided (n = 18; 54.5%), with an average size of 3.46 ± 1.71 cm. The most common location was cisternal (n = 11; 33%), followed by cavernous sinus (CS) proper (n = 9; 27%), cisterocavernous (n = 8; 24%), orbital (n = 4; 12%) and caverno-orbital (n = 1; 3%). CN VI recovery was reported in less than half the cohort (n = 14; 45%). Tumor extension in the CS was significantly associated with lesser likelihood (odds ratio [OR], 0.07; 95% confidence interval [CI], 0.01-0.98; P = 0.048) of postsurgical CN VI recovery. Although female gender (OR, 0.86; 95% CI, 0.07-10.09; P = 0.906), large tumor size (>2.5 cm) (OR, 0.45; 95% CI, 0.07-2.89; P = 0.397), and solid consistency (OR, 0.37; 95% CI, 0.03-4.19; P = 0.421) were associated with lesser odds for recovery, these were not statistically significant. Likewise, although gross total resection (OR, 6.28; 95% CI, 0.33-118.25; P = 0.220) was associated with higher odds of nerve recovery, the estimates were statistically insignificant. CONCLUSIONS: CS involvement is associated with lesser odds for functional nerve recovery in patients undergoing surgical resection for CN VI schwannoma.

Moebius syndrome: clinical features, diagnosis, management and early intervention.

BACKGROUND: Moebius syndrome (MBS) is rare disease characterized by nonprogressive congenital uni- or bi-lateral facial (i. e. VII cranial nerve) and abducens (i. e. VI cranial nerve) palsy. Although the neurological and ophthalmological findings are quite well-known, data concerning the attendant functional difficulties and their changes over time are seldom addressed. In this study we attempt to estimate the prevalence of clinical and functional data in an Italian cohort affected by MBS. METHODS: The study included 50 children, 21 males and 29 females, aged 1 month to 14 years. The patients entered into a multidisciplinary diagnostic and follow-up protocol that had the specific purpose of detecting clinical and developmental deficits related to MBS. RESULTS: Involvement of the VII cranial nerve (total/partial, bilateral or unilateral) was present in 96 % of patients, and of the VI nerve in 85 %. Two patients were without impairment of the VII nerve and seven patients had no involvement of the VI nerve and were thus classified as Moebius-like because of the involvement of other CNs. Additional affected CNs were numbers III-IV in 16 %, V in 11 %, VIII and X each in 8 %, the XI in 6 %, the IX, most often partially, in 22 %, and the XII in 48 % of cases. Their development was characterized by global delay at one year of age, motor, emotional and speech difficulties at two years of age, a trend toward normalization at three years of age but with weakness in hand-eye coordination, and achieving average results at five years of age. Overall 90 % of children had a normal developmental quotient whereas only 10 % manifested cognitive deficits. CONCLUSION: Early rehabilitation may enhance the recovery of normal function, particularly in vulnerable areas of development. It is possible that early intervention that integrates sensory and visual information with emotional difficulties can improve the prognosis of the child with MBS.

Motor unit number estimation as a complementary test to routine electromyography in the diagnosis of amyotrophic lateral sclerosis.

Electromyographic (EMG) abnormalities that reveal denervation and reinnervation caused by lower motor neuron degeneration do not reflect the number of motor units that determines muscle strength. Consequently, motor unit activity potential (MUAP) parameters do not reflect muscle dysfunction. The aim of the study was to compare the value of motor unit number estimation (MUNE) and MUAP parameters as indicators of clinical muscle dysfunction in patients with amyotrophic lateral sclerosis (ALS), and to analyze the role of MUNE as a supplement to the EMG criteria for the diagnosis of ALS. In 25 patients with ALS, MUNE by the multipoint incremental method in the abductor digiti minimi (ADM) and quantitative EMG in the first dorsal interosseous (FDI) were obtained. The Medical Research Council (MRC) scale was used to evaluate clinical muscle dysfunction. A strong correlation between the number of motor units evaluated by MUNE and ADM clinical function by the MRC scale was found (P<0.001). An increased value of surface-detected single motor action potential was associated with a decreased MRC score for ADM (P<0.1). No relation was found between MUAP parameters in FDI and MRC scores. Our data support the value of the MUNE method for the detection of motor unit loss in ALS, and it could be postulated that MUNE studies may be considered complementary tests for ALS in a future revision of ALS criteria.

Strabismus.

Defining the type of strabismus creates a framework for work-up and management. Comitant esotropia is most commonly a childhood condition treated with glasses and surgery. Comitant exotropia is often a childhood condition that may require surgical correction. Microvascular disease is the most common cause of ocular cranial nerve palsies in adult patients.

Presumed canine trigemino-abducens synkinesis in a dog.

A ten-year-old male neutered Rhodesian ridgeback cross dog was presented for the investigation of abnormal bilateral protrusion of the third eyelid when chewing. Physical, ophthalmological, and neurological examinations were unremarkable. Thoracic radiographs, abdominal ultrasound, and magnetic resonance of the brain and orbits failed to reveal any abnormalities. Cerebrospinal fluid analysis revealed elevated protein, but the nucleated cell count was normal. trigemino-abducens synkinesis was presumptively diagnosed. Aetiopathogenesis of this condition is discussed. To the authors' knowledge, this is the first report of presumed trigemino-abducens synkinesis in a dog.

Sixth nerve palsy associated with obstruction in Dorello's canal, accompanied by nodular type muscular sarcoidosis.

A 52-year-old Japanese woman complaining of horizontal double vision for 10 days was admitted to our hospital. Neurological examination revealed left abducent nerve palsy and muscle swelling in her thighs. Brain MRI showed obstruction in the spinal fluid space of the left Dorello's canal, which transmits a portion of the abducent nerve. In Ga-67-enhanced citrate scintigraphy, wide accumulation was seen in her bilateral thighs, lower legs, and gluteus muscles. Muscular MRI showed a star-shaped central structure on short tau inversion recovery (STIR) images, and the three stripes sign on T2-weighted images. These MRI findings indicated nodular-type muscular sarcoidosis. A muscle biopsy from the quadriceps femoris showed granulomatous epithelioid giant cells and non-necrotizing chronic lymphadenitis, which also indicate sarcoidosis. Her condition was considered to be caused by sarcoid granulomas obstructing Dorello's canal. She was treated with oral prednisolone (1 mg·kg(-1)·day(-1)) and her symptoms and MRI findings improved. This is the first known report of abducent nerve impairment in Dorello's canal, other than fetal hypoplasia. Brain MRI, muscular MRI, and muscle biopsy are useful for the diagnosis of abducent nerve palsy, and it is important to consider Dorello's canal obstruction by sarcoidosis. Complete remission can be achieved with proper treatment.

Paresia del vi par como primera manifestación de la DM Tipo 2 / VI cranial nerve palsy as a debut of DM type 2

Caso Clínico. Varón de 56 años sin antecedentes personales de interés, que acude a urgencias por diplopia brusca en la mirada horizontal. A la exploración oftalmológica sólo destaca una restricción a la levoversión en el ojo izquierdo (OI), sin alteraciones en la visión biomicroscopía o fundoscopia. La exploración neurológica y el TAC eran normales. No refería otros síntomas, pero en la analítica observamos una glucemia esporádica de 298 mg/dl y una HbA1c 11,7%, criterio diagnóstico de diabetes (DM) tipo 2, de la que fue tratado. Discusión. La DM es una enfermedad metabólica con alto riesgo cardiovascular, en especial para aquellos enfermos con factores de riesgo asociados. Mientras que la DM tipo 1 presenta sintomatología desde su comienzo diagnóstico, la tipo 2 puede pasar desapercibida. Las complicaciones agudas a destacar para estos enfermos son la cetoacidosis y el estado hiperglucémico hiperosmolar. Sin embargo la neuropatía periférica se trata de un infrecuente síntoma debut (AU)

Case Report. 56 year old male with no relevant past medical history. He came to the emergency department with sudden horizontal dyplopia. Ophthalmological examination only revealed a levoversion restriction in his left eye (OS), with no changes in his vision acuity, slit lamp examination or funduscopy. Neurological examination and CT scan were normal. He had no other symptoms, but the analytical observed glucose levels of 298 mg / dl and HbA1c 11.7%, which is a diagnostic criteria for diabetes (DM) type 2, and so he was treated. Discussion. DM is a metabolic disease with high cardiovascular risk, especially for those patients with associated risk factors. While diabetes type 1 has symptoms just after diagnosis, onset type 2 may go unnoticed. Acute complications are ketoacidosis and hyperosmolar hyperglycemic state, however peripheral neuropathy is an uncommon symptom debut (AU)

Does infantile abduction deficit indicate duane retraction syndrome until disproven?

Duane retraction syndrome consists of abduction deficit and palpebral fissure narrowing, upshoots, or downshoots on adduction. Infants with abduction deficit should be considered to have Duane retraction syndrome until disproven, because congenital abducens nerve palsy is extremely rare. The abducens nerve on the affected side is absent in type 1 Duane retraction syndrome and in some type 3 patients. The authors present a 7-month-old girl who showed limitation of abduction simulating Duane retraction syndrome. High-resolution magnetic resonance imaging (MRI) revealed atrophic lateral rectus and present abducens nerve. This report is important because this case showed that congenital abducens nerve palsy exists, although it is extremely rare, and high-resolution MRI could be pivotal for the differentiation of Duane retraction syndrome and congenital abducens nerve palsy in infancy.

Delayed diagnosis of lyme neuroborreliosis presenting with abducens neuropathy without intrathecal synthesis of Borrelia antibodies.

Lyme borreliosis is the most common tick-born infection in Europe. Global climate change expanding the range of tick vectors and an increase in the incidence suggest that this disease will remain an important health issue in the forthcoming decades. Lyme borreliosis is a multisystem organ disorder affecting the nervous system in 10% to 15% of cases. Lyme neuroborreliosis can present with any disorder of the central and peripheral nervous systems. The neuro-ophthalmological manifestations are a rare feature of the disease. The intrathecal synthesis of Borrelia burgdorferi antibodies is of diagnostic importance, but in rare cases, immunoglobulins against the Borrelia burgdorferi antigen may not be detected. We report a case of possible Lyme neuroborreliosis presenting with sixth cranial nerve neuropathy at the onset of the disease further developing into typical meningoradiculitis and multiple mononeuropathy. Surprisingly, Borrelia burgdorferi antibodies were not detected in the cerebrospinal fluid.

Contralateral lateral rectus recession versus recess-resect for recurrent exotropia after unilateral recess-resect.

BACKGROUND: To compare outcomes following contralateral lateral rectus (LR) recession and recess-resect (RR) procedures for recurrent exotropia of 20-25 prism dioptres (PD) after unilateral RR. METHODS: 39 subjects were included in this retrospective study. All underwent, as a primary surgery for intermittent exotropia, unilateral RR on the non-dominant eye. They were assigned to the subsequent contralateral LR recession (LR, n=19) or RR (n=20) group for recurrent exotropia of 20-25 PD. Surgical success was defined as alignment between 5 PD esodeviation and 10 PD exodeviation. RESULTS: The mean follow-up duration after the reoperation was 32.3±26.4 months in the LR group and 30.5±26.8 in the RR group (p=0.945). The mean deviation angles at postoperative 1 day were -0.7 PD (overcorrection) in the LR group and -4.3 PD in the RR group (p=0.047). The deviation angles at 3 and 6 months postoperatively were not significant (p=0.771, p=0.923). The final successful outcome was achieved in 63.2% of patients in the LR group and in 65% of patients in the RR group (p=0.905). CONCLUSIONS: Contralateral LR recession was found to be a safe and effective procedure for the treatment of recurrent exotropia of 20-25 PD after unilateral RR for intermittent exotropia. With LR recession, the intentional overcorrection in the immediate postoperative period could be avoided.

Comparison of primary position measurements and abduction deficit between type 1 Duane syndrome and sixth cranial nerve palsy.

BACKGROUND: Unilateral Duane retraction syndrome type 1 (DRS-I) and unilateral sixth nerve palsy (6NP) present with limitation of abduction, incomitant esotropia, and frequently, a compensatory head turn. The purpose of this study was to compare the mean primary position measurement and to correlate this with the abduction deficit to determine if these measurements may be used to differentiate between the 2 conditions when other clinical signs of DRS-I (globe retraction, changes in lid fissure height, and upshoots/downshoots) are subtle. METHODS: A database search of patients examined over a 5-year period revealed 69 cases of DRS-I and 62 cases of unilateral 6NP. Primary position measurements both at distance and near and limitation of abduction on version testing were recorded and compared. RESULTS: Mean abduction deficit was -3.5 ± 0.1 for DRS-I and -2.6 ± 0.2 for 6NP (P = 0.0004). Mean esotropia at near was 8.4 ± 1.1 prism diopters (PD) for DRS-I and 27.2 ± 2.4 PD for 6NP (P < 0.0001). Mean esotropia at distance was 10.3 ± 1.3 PD for DRS-I and 36.4 ± 2.4 PD for 6NP (P < 0.0001). The mean distance-near disparity for DRS-I was 1.94 ± 0.62 PD and 9.19 ± 1.28 PD for 6NP (P < 0.0001). The age-group of ≤2 years consisted of 23 DRS-I and only 2 6NP cases. The age-group between >2 years and <18 years had 41 DRS-I and 16 6NP cases, respectively. Finally, the age-group of ≥18 years had only 5 DRS-I and 44 6NP cases (P < 0.0001). CONCLUSION: Patients with DRS-I showed greater abduction deficit yet significantly less esotropia in primary position than those with 6NP. Patients with 6NP were more likely to have a significant distance-near disparity. In addition, patients with DRS-I tended to be younger than those with 6NP. This report documents that DRS-I and 6NP can be differentiated based on magnitude of primary position esotropia, comparison of primary position esotropia with severity of abduction deficit, distance-near disparity, and patient age.

[Intraoperative identification of oculomotor, trochlear and abducent nerves in surgery of invasive cranioorbital tumors (new technique)].

BACKGROUND: Aim of the study was to evaluate effectiveness of intraoperative identification of oculomotor nerves (OMN) in resection of skull base tumors invading superior orbital fissure and cavernous sinus. MATERIALS AND METHODS: 69 patients with cranioorbital tumors operated in Burdenko Neurosurgical Institute (Moscow, Russia) since 2000 until 2005 were included in the study. They were divided into 2 groups: 19 patients treated with intraoperative identification of OMN and 50 patients in the control group. Craniorbital meningiomas were in the majority among all cases. Intraoperative identification of OMN was performed using coaxial electrode while muscular response was registered through electrodes inserted in m. levator palpebrae superioris, m. obliquus superior and m. rectus lateralis (for III, IV and VI cranial nerves, respectively). Identification of IMN trunci was repeated throughout the whole stage of tumor resection for their preservation. RESULTS: comparison of dynamics of oculomotor dysfunction in early postoperative period in patients of both groups demonstrated that intraoperative identification of OMN allowed to decrease the frequency of oculomotor deficit. The rates in main and control groups were: for III and IV nerves--37% and 68% (p < 0.05), for VI nerve--47% and 54% (p > 0.05), respectively. CONCLUSION: application of intraoperative identification of OMN allows to decrease the risk of oculomotor deterioration due to III and IV nerve dysfunction by 1.8 times. Technically the method is quite simple and not time-consuming procedure.