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Italian Journal of Pediatrics Jun 2016Moebius 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...
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.
Topics: Abducens Nerve; Adolescent; Child; Child, Preschool; Cohort Studies; Disability Evaluation; Early Medical Intervention; Facial Nerve; Female; Follow-Up Studies; Humans; Infant; Infant, Newborn; Italy; Male; Mobius Syndrome; Neuropsychological Tests; Rare Diseases; Risk Assessment; Treatment Outcome
PubMed: 27260152
DOI: 10.1186/s13052-016-0256-5 -
The Kurume Medical Journal Mar 2022An ossified left petroclinoid ligament was found during routine dissection of the skull base in an adult male cadaver. The petroclinoid ligament is clinically and... (Review)
Review
An ossified left petroclinoid ligament was found during routine dissection of the skull base in an adult male cadaver. The petroclinoid ligament is clinically and surgically important given its anatomical relationships to cranial nerves III, V, and VI, so its ossification is a risk factor for injuries due to trauma, increased intracranial pressure, and vascular and tumor compression resulting in abducens and oculomotor palsies. The causes of petroclinoid ligament ossification are yet to be completely elucidated although several reports have associated them with age-related and physiological processes. Assessing the integrity of the petroclinoid ligament is important during skull base surgical interventions to avoid postoperative complications. Therefore, this paper reviews the petroclinoid ligament and its variation, the ossified petroclinoid ligament.
Topics: Abducens Nerve; Adult; Cadaver; Dissection; Humans; Ligaments; Male; Osteogenesis
PubMed: 35095019
DOI: 10.2739/kurumemedj.MS671007 -
Northern Clinics of Istanbul 2022Abducens nerve paralysis is the most common ocular motor neuropathy. In this article, we aimed to compare the causes of isolated abducens nerve palsy in terms of...
OBJECTIVE
Abducens nerve paralysis is the most common ocular motor neuropathy. In this article, we aimed to compare the causes of isolated abducens nerve palsy in terms of demographic, clinical features, and prognosis.
METHODS
Thirty-six isolated abducens nerve palsy patients were prospectively enrolled in the study. The demographic, clinical features, and prognosis compared in two etiological groups as microvascular and other causes.
RESULTS
The most common etiology was microvascular, which was seen in 16/36 (44.4%) patients. Mean clinical recovery time was 2.5±1.3 months (range, 10 days-6 months). When etiological groups were compared as microvascular and other causes, the mean age of the microvascular group was significantly higher (62.8±13.3 vs. 44.5±16.4, p=0.001). Diabetes mellitus was seen significantly higher in the microvascular group than other causes group (p=0.001), but no significant difference was observed in terms of other atherosclerotic risk factors (p>0.05). The fasting blood glucose and hemoglobin A1c value were significantly higher in the microvascular group (p=0.02 and p=0.02, respectively). There was no significant difference in terms of clinical improvement and clinical recovery times between groups (p>0.05).
CONCLUSION
There is no difference between microvascular group and other causes in terms of clinical outcome, while the mean age and presence of diabetes were higher in the microvascular group. The presence of diabetes should be questioned in cases with isolated abducens nerve palsy.
PubMed: 36276565
DOI: 10.14744/nci.2021.15483 -
The Journal of International Medical... Jan 2021Our aim was to explore the clinical characteristics and diagnostic methods of benign abducens nerve palsy in children. The clinical and laboratory characteristics,...
Our aim was to explore the clinical characteristics and diagnostic methods of benign abducens nerve palsy in children. The clinical and laboratory characteristics, treatment approach, and prognosis of two children with benign abducens nerve palsy were retrospectively evaluated. The main clinical symptoms of the two children were limited binocular movement accompanied by double vision, and the positive physical signs were limited binocular abduction accompanied by diplopia. No abnormalities were found in laboratory examinations or in imaging of the head, chest, and abdomen. Both children were treated with B vitamins, methylprednisolone, and gamma globulin, and the clinical symptoms disappeared within 3 months of starting treatment. The cause of benign abducens nerve palsy in children is unknown, but may be related to immune factors. In the two cases presented here, the patients recovered after treatment with immunomodulators.
Topics: Abducens Nerve Diseases; Child; Diplopia; Follow-Up Studies; Humans; Prognosis; Retrospective Studies
PubMed: 33445999
DOI: 10.1177/0300060520986702 -
Internal Medicine (Tokyo, Japan) Oct 2022The most common neurological symptom of spontaneous intracranial hypotension (SIH) is abducens nerve paresis, and the precise pathophysiology is unclear. The accepted...
The most common neurological symptom of spontaneous intracranial hypotension (SIH) is abducens nerve paresis, and the precise pathophysiology is unclear. The accepted explanation is traction on the cranial nerves caused by the downward displacement of the cranial content. We herein report magnetic resonance imaging of SIH that can explain the mechanism underlying abducens nerve paresis. The cavernous sinuses were particularly thickened compared with the surrounding dura. This phenomenon can be explained by venous swelling, which can occur after leakage of cerebrospinal fluid in a closed cavity. This swelling pushes the abducens nerve up, which then causes abducens nerve paresis.
Topics: Abducens Nerve; Abducens Nerve Diseases; Cavernous Sinus; Edema; Humans; Intracranial Hypotension; Magnetic Resonance Imaging; Paresis
PubMed: 35342130
DOI: 10.2169/internalmedicine.8488-21 -
Clinical Ophthalmology (Auckland, N.Z.) 2022Abduction limitation in esotropic Duane retraction syndrome (DRS), esotropic Mobius syndrome, and sixth nerve palsy is one of the difficult-to-manage problems in... (Review)
Review
Abduction limitation in esotropic Duane retraction syndrome (DRS), esotropic Mobius syndrome, and sixth nerve palsy is one of the difficult-to-manage problems in strabismus surgery. The procedure of superior rectus transposition (SRT) was introduced by Johnston et al. In this procedure, the superior rectus (SR) muscle is disinserted and sutured adjacent to the insertion of lateral rectus (LR) muscle. The purpose of this review is to explore literature about efficacy and safety of SRT and its usage in strabismus surgery.
PubMed: 36444206
DOI: 10.2147/OPTH.S359313 -
Postgraduate Medical Journal Sep 2000Gradenigo's syndrome, which is characterised by the triad of suppurative otitis media, pain in the distribution of the trigeminal nerve, and abducens nerve palsy may... (Review)
Review
Gradenigo's syndrome, which is characterised by the triad of suppurative otitis media, pain in the distribution of the trigeminal nerve, and abducens nerve palsy may give rise to potentially fatal complications. Knowledge of the aetiology and appropriate investigations can lead to early diagnosis. A case is reported which illustrates this.
Topics: Abducens Nerve Diseases; Aged; Humans; Male; Otitis Media, Suppurative; Syndrome; Trigeminal Neuralgia
PubMed: 10964120
DOI: 10.1136/pmj.76.899.559 -
Brain Structure & Function Sep 2021Extraocular motoneurons initiate dynamically different eye movements, including saccades, smooth pursuit and vestibulo-ocular reflexes. These motoneurons subdivide into...
Extraocular motoneurons initiate dynamically different eye movements, including saccades, smooth pursuit and vestibulo-ocular reflexes. These motoneurons subdivide into two main types based on the structure of the neuro-muscular interface: motoneurons of singly-innervated (SIF), and motoneurons of multiply-innervated muscle fibers (MIF). SIF motoneurons are thought to provoke strong and brief/fast muscle contractions, whereas MIF motoneurons initiate prolonged, slow contractions. While relevant for adequate functionality, transmitter and ion channel profiles associated with the morpho-physiological differences between these motoneuron types, have not been elucidated so far. This prompted us to investigate the expression of voltage-gated potassium, sodium and calcium ion channels (Kv1.1, Kv3.1b, Nav1.6, Cav3.1-3.3, KCC2), the transmitter profiles of their presynaptic terminals (vGlut1 and 2, GlyT2 and GAD) and transmitter receptors (GluR2/3, NMDAR1, GlyR1α) using immunohistochemical analyses of abducens and trochlear motoneurons and of abducens internuclear neurons (INTs) in macaque monkeys. The main findings were: (1) MIF and SIF motoneurons express unique voltage-gated ion channel profiles, respectively, likely accounting for differences in intrinsic membrane properties. (2) Presynaptic glutamatergic synapses utilize vGlut2, but not vGlut1. (3) Trochlear motoneurons receive GABAergic inputs, abducens neurons receive both GABAergic and glycinergic inputs. (4) Synaptic densities differ between MIF and SIF motoneurons, with MIF motoneurons receiving fewer terminals. (5) Glutamatergic receptor subtypes differ between MIF and SIF motoneurons. While NMDAR1 is intensely expressed in INTs, MIF motoneurons lack this receptor subtype entirely. The obtained cell-type-specific transmitter and conductance profiles illuminate the structural substrates responsible for differential contributions of neurons in the abducens and trochlear nuclei to eye movements.
Topics: Abducens Nerve; Animals; Eye Movements; Ion Channels; Macaca; Motor Neurons; Oculomotor Muscles; Reflex, Vestibulo-Ocular; Trochlear Nerve
PubMed: 34181058
DOI: 10.1007/s00429-021-02315-7 -
Journal Francais D'ophtalmologie Dec 2022
Topics: Humans; Abducens Nerve; Abducens Nerve Diseases; COVID-19; COVID-19 Vaccines
PubMed: 36244866
DOI: 10.1016/j.jfo.2022.04.003