Cranial Nerves ; Humans ; Lymphoma ; Nervous System Malformations

Upon review, it is noted that recurrent painful ophthalmoplegic neuropathy (RPON) is a rare neurological syndrome characterized by recurrent unilateral headaches and painful ophthalmoplegia of the ipsilateral oculomotor nerve. As seen on brain MRI, thickening and enhancement of the oculomotor cranial nerve can be observed in these cases. We experienced a case of RPON in an adult patient who showed thickening and enhancement of the oculomotor nerve on gadolinium-enhanced 3D-FLAIR image. The authors report a case of RPON with a review of the literature.
Adult ; Brain ; Cranial Nerves ; Headache ; Humans ; Magnetic Resonance Imaging ; Oculomotor Nerve ; Oculomotor Nerve Diseases ; Ophthalmoplegia ; Paralysis

Möbius syndrome is a rare congenital condition, characterized by abducens and facial nerve palsy, resulting in limitation of lateral gaze movement and facial diplegia. However, to our knowledge, there have been few studies on evaluation of cranial nerves, on MR imaging in Möbius syndrome. Herein, we describe a rare case of Möbius syndrome representing limitation of lateral gaze, and weakness of facial expression, since the neonatal period. In this case, high-resolution MR imaging played a key role in diagnosing Möbius syndrome, by direct visualization of corresponding cranial nerves abnormalities.
Cranial Nerves ; Facial Expression ; Facial Nerve ; Magnetic Resonance Imaging ; Paralysis

PURPOSE: To understand the ophthalmic clinical features and outcomes of facial nerve palsy patients who were referred to an ophthalmic clinic for various conditions like Bell's palsy, trauma, and brain tumor. METHODS: A retrospective study was conducted of 34 eyes from 31 facial nerve palsy patients who visited a clinic between August 2007 and July 2017. The clinical signs, management, and prognosis were analyzed. RESULTS: The average disease period was 51.1 ± 20.6 months, and the average follow-up duration was 24.0 ± 37.5 months. The causes of facial palsy were as follows: Bell's palsy, 13 cases; trauma, six cases; brain tumor, five cases; and cerebrovascular disease, four cases. The clinical signs were as follows: lagophthalmos, 24 eyes; corneal epithelial defect, 20 eyes; conjunctival injection, 19 eyes; ptosis, 15 eyes; and tearing, 12 eyes. Paralytic strabismus was found in seven eyes of patients with another cranial nerve palsy (including the third, fifth, or sixth cranial nerve). Conservative treatments (like ophthalmic ointment or eyelid taping) were conducted along with invasive procedures (like levator resection, tarsorrhaphy, or botulinum neurotoxin type A injection) in 17 eyes (50.0%). Over 60% of the patients with symptomatic improvement were treated using invasive treatment. At the time of last following, signs had improved in 70.8% of patients with lagophthalmos, 90% with corneal epithelium defect, 58.3% with tearing, and 72.7% with ptosis. The rate of improvement for all signs was high in patients suffering from facial nerve palsy without combined cranial nerve palsy. CONCLUSIONS: The ophthalmic clinical features of facial nerve palsy were mainly corneal lesion and eyelid malposition, and their clinical course improved after invasive procedures. When palsy of the third, fifth, or sixty cranial nerve was involved, the prognosis and ophthalmic signs were worse than in cases of simple facial palsy. Understanding these differences will help the ophthalmologist take care of patients with facial nerve palsy.
Bell Palsy ; Brain Neoplasms ; Cerebrovascular Disorders ; Cranial Nerve Diseases ; Cranial Nerves ; Epithelium, Corneal ; Eyelids ; Facial Nerve ; Facial Paralysis ; Follow-Up Studies ; Humans ; Paralysis ; Prognosis ; Retrospective Studies ; Strabismus ; Tears

BACKGROUND AND PURPOSE: The most-common initial manifestation of Miller Fisher syndrome (MFS) is diplopia due to acute ophthalmoplegia. However, few studies have focused on ocular motility findings in MFS. This study aimed to determine the pattern of extraocular muscle (EOM) paresis in MFS patients. METHODS: We consecutively recruited MFS patients who presented with ophthalmoplegia between 2010 and 2015. The involved EOMs and the strabismus pattern in the primary position were analyzed. Antecedent infections, other involved cranial nerves, and laboratory findings were also reviewed. We compared the characteristics of the patients according to the severity of ophthalmoplegia between complete ophthalmoplegia (CO) and incomplete ophthalmoplegia (IO). RESULTS: Twenty-five patients (15 males and 10 females) with bilateral ophthalmoplegia were included in the study. The most-involved and last-to-recover EOM was the lateral rectus muscle. CO and IO were observed in 11 and 14 patients, respectively. The patients were aged 59.0±18.4 years (mean±SD) in the CO group and 24.9±7.4 years in the IO group (p<0.01), and comprised 63.6% and 21.4% females, respectively (p=0.049). Elevated cerebrospinal fluid protein was identified in 60.0% of patients with CO and 7.7% of patients with IO (p=0.019) for a mean follow-up time from the initial symptom onset of 3.7 days. CONCLUSIONS: The lateral rectus muscle is the most-involved and last-to-recover EOM in ophthalmoplegia. The CO patients were much older and were more likely to be female and have an elevation of cerebrospinal fluid protein than the IO patients.
Cerebrospinal Fluid ; Cranial Nerves ; Diplopia ; Female ; Follow-Up Studies ; Guillain-Barre Syndrome ; Humans ; Jupiter ; Male ; Miller Fisher Syndrome ; Ophthalmoplegia ; Paresis ; Strabismus

No abstract available.
Cranial Nerve Diseases ; Cranial Nerves ; Hoarseness ; Vertebrobasilar Insufficiency ; Vocal Cord Paralysis

Osteopetrosis is characterized by impaired osteoclast function and increased bone density. Infantile osteopetrosis is a severe form of the disease and has characteristics such as diffusely sclerotic skeleton, pancytopenia, cranial nerve entrapment, infection susceptibility, and abnormal craniofacial appearance. Patients with infantile osteopetrosis often experience developmental delay, and may have a short life span.A 14-month-old girl with osteopetrosis presented to the department of pediatric dentistry. Incipient caries on deciduous incisors were observed. The patient revisited 4 years of age. Besides medical problems, oral complications such as growth retardation, narrow upper arch, crowding, dental caries, and abnormal tooth development were observed. After consultation with her pediatrician, dental treatments were performed on the deciduous molars under sedation after a prophylactic antibiotic injection. At a periodic follow-up, multiple deciduous teeth were treated and extracted, and oral-rehabilitation with a removable partial denture was initiated.Patient with osteopetrosis are highly susceptible to infection because of their compromised immune system and problems associated with wound healing that lead to osteomyelitis or sepsis development.Active participation in dental care for sugar intake management and proper oral hygiene are obligatory.
Bone Density ; Cranial Nerves ; Crowding ; Dental Care ; Dental Caries ; Denture, Partial, Removable ; Female ; Follow-Up Studies ; Humans ; Immune System ; Incisor ; Infant ; Molar ; Oral Hygiene ; Osteoclasts ; Osteomyelitis ; Osteopetrosis ; Pancytopenia ; Pediatric Dentistry ; Sepsis ; Skeleton ; Tooth ; Tooth, Deciduous ; Wound Healing

Intraoperative monitoring (IOM) utilizes electrophysiological techniques as a surrogate test and evaluation of nervous function while a patient is under general anesthesia. They are increasingly used for procedures, both surgical and endovascular, to avoid injury during an operation, examine neurological tissue to guide the surgery, or to test electrophysiological function to allow for more complete resection or corrections. The application of IOM during pediatric brain tumor resections encompasses a unique set of technical issues. First, obtaining stable and reliable responses in children of different ages requires detailed understanding of normal ageadjusted brain-spine development. Neurophysiology, anatomy, and anthropometry of children are different from those of adults. Second, monitoring of the brain may include risk to eloquent functions and cranial nerve functions that are difficult with the usual neurophysiological techniques. Third, interpretation of signal change requires unique sets of normative values specific for children of that age. Fourth, tumor resection involves multiple considerations including defining tumor type, size, location, pathophysiology that might require maximal removal of lesion or minimal intervention. IOM techniques can be divided into monitoring and mapping. Mapping involves identification of specific neural structures to avoid or minimize injury. Monitoring is continuous acquisition of neural signals to determine the integrity of the full longitudinal path of the neural system of interest. Motor evoked potentials and somatosensory evoked potentials are representative methodologies for monitoring. Free-running electromyography is also used to monitor irritation or damage to the motor nerves in the lower motor neuron level : cranial nerves, roots, and peripheral nerves. For the surgery of infratentorial tumors, in addition to free-running electromyography of the bulbar muscles, brainstem auditory evoked potentials or corticobulbar motor evoked potentials could be combined to prevent injury of the cranial nerves or nucleus. IOM for cerebral tumors can adopt direct cortical stimulation or direct subcortical stimulation to map the corticospinal pathways in the vicinity of lesion. IOM is a diagnostic as well as interventional tool for neurosurgery. To prove clinical evidence of it is not simple. Randomized controlled prospective studies may not be possible due to ethical reasons. However, prospective longitudinal studies confirming prognostic value of IOM are available. Furthermore, oncological outcome has also been shown to be superior in some brain tumors, with IOM. New methodologies of IOM are being developed and clinically applied. This review establishes a composite view of techniques used today, noting differences between adult and pediatric monitoring.
Adult ; Anesthesia, General ; Anthropometry ; Brain Neoplasms ; Brain ; Child ; Cranial Nerves ; Electromyography ; Evoked Potentials, Auditory, Brain Stem ; Evoked Potentials, Motor ; Evoked Potentials, Somatosensory ; Humans ; Infratentorial Neoplasms ; Intraoperative Neurophysiological Monitoring ; Longitudinal Studies ; Monitoring, Intraoperative ; Motor Neurons ; Muscles ; Neurophysiology ; Neurosurgery ; Peripheral Nerves ; Prospective Studies

Ramsay Hunt syndrome is a type of acute herpes zoster, which occurs by reactivation of the varicella-zoster virus at the geniculate ganglion. Clinical presentation of Ramsay Hunt syndrome includes a vesicular rash on the ear (herpes zoster oticus) or in the oral mucosa accompanied by acute peripheral facial nerve paralysis. Other cranial nerves such as V, IX, XI, and XII are often involved. Additional variability of the clinical picture of Ramsay Hunt syndrome is produced by varying patterns of skin involvement explained by individual anastomoses between cranial and cervical nerves. Combination treatment containing anti-viral agents and steroids is recommended for the treatment of Ramsay Hunt syndrome. Additionally, early diagnosis of Ramsay Hunt syndrome is a crucial factor to improve damaged nerves in Ramsay Hunt syndrome, which initiates treatment as soon as possible.
Cranial Nerves ; Ear ; Early Diagnosis ; Exanthema ; Facial Nerve ; Facial Paralysis ; Geniculate Ganglion ; Herpes Zoster ; Herpes Zoster Oticus ; Herpesvirus 3, Human ; Mouth Mucosa ; Paralysis ; Skin ; Steroids

BACKGROUND: Herpes zoster of the head and neck commonly presents with Ramsay Hunt syndrome. However, vesicular eruptions may occur on the pharyngeal or laryngeal area with multiple lower cranial-nerve (CN) palsy. CASE REPORT: We report on the case of a 54-year-old man with herpes zoster of the pharynx and larynx with multiple CN palsy and persistent hiccups. He initially developed progressive dysphagia, hoarseness, and persistent hiccups (CN IX and X). After admission, Dizziness, hearing impairment, and peripheral facial palsy (CN VII and VIII) were complicated. The results of a polymerase chain reaction test of saliva and vesicular fluid from the ear and throat were strongly positive for varicella zoster virus. The progression of CN palsy was in an ascending sequence. CONCLUSION: We suggest that the sequence of CN palsy may be either ascending or descending, depending on the initial site of involvement.
Cranial Nerve Diseases ; Cranial Nerves ; Deglutition Disorders ; Dizziness ; Ear ; Facial Paralysis ; Head ; Hearing Loss ; Herpes Zoster Oticus ; Herpes Zoster ; Herpesvirus 3, Human ; Hiccup ; Hoarseness ; Humans ; Laryngitis ; Larynx ; Middle Aged ; Neck ; Paralysis ; Pharynx ; Polymerase Chain Reaction ; Saliva