-
Neurology India 2023Primary hypophysitis is a rare condition, usually diagnosed retrospectively after surgery for a suspected pituitary adenoma. Improved awareness of the condition and... (Review)
Review
BACKGROUND
Primary hypophysitis is a rare condition, usually diagnosed retrospectively after surgery for a suspected pituitary adenoma. Improved awareness of the condition and better imaging have resulted in more patients being diagnosed without surgery.
OBJECTIVE
A retrospective chart analysis study of hypophysitis from a single secondary endocrine and neurosurgical referral center in eastern India was conducted between 1999 and 2021 to assess the diagnostic and therapeutic challenges posed by these patients.
METHODS AND MATERIAL
Fourteen patients presented to the center between 1999 and 2021. All patients had an MRI of the head with contrast and a full clinical workup. Twelve patients had headache, of whom one had progressive visual impairment. One patient had severe weakness, attributed later to hypoadrenalism and one had sixth nerve palsy.
RESULTS
Six patients had glucocorticoids as their primary treatment, four refused treatment, and one was on glucocorticoid replacement. One patient had decompressive surgery because of progressive visual loss, and two had surgery because of a presumptive diagnosis of pituitary adenoma. There was no difference between the patients who had glucocorticoids and those who did not.
CONCLUSIONS
Our data suggest that it is possible to identify most patients with hypophysitis on clinical and radiological grounds. In the largest published series on this subject and in ours, glucocorticoid treatment did not alter the outcome.
Topics: Humans; Glucocorticoids; Pituitary Neoplasms; Retrospective Studies; Abducens Nerve Diseases; Hypophysitis
PubMed: 36861584
DOI: 10.4103/0028-3886.370481 -
Eye and Brain 2021Vestibular schwannomas (VSs), also called acoustic neuromas, are benign intracranial neoplasms of the vestibulocochlear (VIII) cranial nerve. Management options include... (Review)
Review
Vestibular schwannomas (VSs), also called acoustic neuromas, are benign intracranial neoplasms of the vestibulocochlear (VIII) cranial nerve. Management options include "wait-and-scan," stereotactic radiosurgery and surgical resection. Due to the proximity of the VIII nerve to the facial (VII) nerve in the cerebello-pontine angle, the VII nerve is particularly vulnerable to the effects of surgical resection. This can result in poor eye closure, lagophthalmos and resultant corneal exposure post VS resection. Additionally, compression from the tumor or resection can cause trigeminal (V) nerve damage and a desensate cornea. The combination of an exposed and desensate cornea puts the eye at risk of serious ocular complications including persistent epithelial defects, corneal ulceration, corneal vascularization, corneal melting and potential perforation. The abducens (VI) nerve can be affected by a large intracranial VS causing raised intracranial pressure (a false localizing sign) or as a result of damage to the VI nerve at the time of resection. Other types of neurogenic strabismus are rare and typically transient. Contralaterally beating nystagmus as a consequence of vestibular dysfunction is common post-operatively. This generally settles to pre-operative levels as central compensation occurs. Ipsilaterally beating nystagmus post-operatively should prompt investigation for post-operative cerebrovascular complications. Papilledema (and subsequent optic atrophy) can occur as a result of a large VS causing raised intracranial pressure. Where papilledema follows surgical resection of a VS, it can indicate that cerebral venous sinus thrombosis has occurred. Poor visual function following VS resection can result as a combination of all these potential complications and is more likely with larger tumors.
PubMed: 34621136
DOI: 10.2147/EB.S272326 -
Biomedicines Feb 2023Various complications of pituitary neuroendocrine tumors (PitNET) are reported, and an intratumor hemorrhage or infarct underlying pituitary apoplexy (PA) represents an... (Review)
Review
Various complications of pituitary neuroendocrine tumors (PitNET) are reported, and an intratumor hemorrhage or infarct underlying pituitary apoplexy (PA) represents an uncommon, yet potentially life-threatening, feature, and thus early recognition and prompt intervention are important. Our purpose is to overview PA from clinical presentation to management and outcome. This is a narrative review of the English-language, PubMed-based original articles from 2012 to 2022 concerning PA, with the exception of pregnancy- and COVID-19-associated PA, and non-spontaneous PA (prior specific therapy for PitNET). We identified 194 original papers including 1452 patients with PA (926 males, 525 females, and one transgender male; a male-to-female ratio of 1.76; mean age at PA diagnostic of 50.52 years, the youngest being 9, the oldest being 85). Clinical presentation included severe headache in the majority of cases (but some exceptions are registered, as well); neuro-ophthalmic panel with nausea and vomiting, meningism, and cerebral ischemia; respectively, decreased visual acuity to complete blindness in two cases; visual field defects: hemianopia, cranial nerve palsies manifesting as diplopia in the majority, followed by ptosis and ophthalmoplegia (most frequent cranial nerve affected was the oculomotor nerve, and, rarely, abducens and trochlear); proptosis (N = 2 cases). Risk factors are high blood pressure followed by diabetes mellitus as the main elements. Qualitative analysis also pointed out infections, trauma, hematologic conditions (thrombocytopenia, polycythemia), Takotsubo cardiomyopathy, and T3 thyrotoxicosis. Iatrogenic elements may be classified into three main categories: medication, diagnostic tests and techniques, and surgical procedures. The first group is dominated by anticoagulant and antiplatelet drugs; additionally, at a low level of statistical evidence, we mention androgen deprivation therapy for prostate cancer, chemotherapy, thyroxine therapy, oral contraceptives, and phosphodiesterase 5 inhibitors. The second category includes a dexamethasone suppression test, clomiphene use, combined endocrine stimulation tests, and a regadenoson myocardial perfusion scan. The third category involves major surgery, laparoscopic surgery, coronary artery bypass surgery, mitral valvuloplasty, endonasal surgery, and lumbar fusion surgery in a prone position. PA in PitNETs still represents a challenging condition requiring a multidisciplinary team from first presentation to short- and long-term management. Controversies involve the specific panel of risk factors and adequate protocols with concern to neurosurgical decisions and their timing versus conservative approach. The present decade-based analysis, to our knowledge the largest so far on published cases, confirms a lack of unanimous approach and criteria of intervention, a large panel of circumstantial events, and potential triggers with different levels of statistical significance, in addition to a heterogeneous clinical picture (if any, as seen in subacute PA) and a spectrum of evolution that varies from spontaneous remission and control of PitNET-associated hormonal excess to exitus. Awareness is mandatory. A total of 25 cohorts have been published so far with more than 10 PA cases/studies, whereas the largest cohorts enrolled around 100 patients. Further studies are necessary.
PubMed: 36979658
DOI: 10.3390/biomedicines11030680 -
Frontiers in Pediatrics 2023This study will list the most common comorbidities of congenital facial nerve palsy and how to detect and treat them, with special attention for ENT-problems such as...
OBJECTIVES
This study will list the most common comorbidities of congenital facial nerve palsy and how to detect and treat them, with special attention for ENT-problems such as hearing loss. Congenital facial nerve palsy is a very rare entity but in UZ Brussels hospital there was a follow-up of 16 children in the last 30 years.
METHODS
Literature review has been done, combined with thorough research of our own series of 16 children with congenital facial nerve palsy.
RESULTS
Congenital facial nerve palsy can be part of a known syndrome, most commonly Moebius syndrome, but can also appear solely. It appears often bilateral and with a severe gradation. In our series, hearing loss is frequently seen in association with congenital facial nerve palsy. Other abnormalities are dysfunction of the abducens nerve, ophthalmological problems, retro- or micrognathism and abnormalities of limbs or heart. The majority of the children in our series underwent radiological imaging (CT and/or MRI): the facial nerve but also the vestibulocochlear nerve and middle and inner ear can be evaluated.
CONCLUSION
A multidisciplinary approach of congenital facial nerve palsy is recommended as it can affect various bodily functions. Radiological imaging needs to be done to acquire additional information that can be useful for diagnostic and therapeutic purposes. Although congenital facial nerve palsy may not be treatable itself, its comorbidities can be treated and improve the quality of life of the affected child.
PubMed: 36891227
DOI: 10.3389/fped.2023.1077238 -
Developmental Medicine and Child... Apr 2020To review orofacial disabilities and their consequences in children with Moebius syndrome (MBS).
AIM
To review orofacial disabilities and their consequences in children with Moebius syndrome (MBS).
METHOD
We retrospectively analysed the records of 32 patients (21 males, 11 females) with non-progressive bilateral facial and abducens palsies who had been examined before 6 months of age.
RESULTS
All facial muscles were severely involved in 17 patients; in the 15 others, partial movements were found in the lower face. Most patients (n=24) were unable to smile. Patients frequently presented with congenital trismus (n=20) and drooling (n=18). Additional palsies involved cranial nerves IX and X (n=18) and XII (n=25). Sucking was absent or weak in 30 patients; swallowing was impaired in 25. During the first month of life, feeding disorders were graded as severe/moderate in 25. Respiratory complications occurred in 17. Severe feeding disorders were associated with congenital trismus (p=0.01) and with cranial nerve IX and X palsy (p=0.01). Growth failure between 1 and 6 months of age, followed by catch-up growth between 6 and 12 months, was observed in 20 patients. Between 2 and 5 years of age, 25 out of 32 patients attained normal oral diet and 28 out of 29 showed normal growth.
INTERPRETATION
Children with MBS frequently require adjusted therapeutic options to prevent failure to thrive. Congenital trismus, cranial nerve IX and X palsy, and laryngeal-tracheal dysfunctions are predictors of severe feeding disorders.
WHAT THIS PAPER ADDS
Moebius syndrome frequently induces reduced oral intake and early failure to thrive. Normal oral diet and growth parameters are attained at 2 to 5 years of age. Congenital trismus, pharyngeal palsy, and laryngeal disorders predict dysphagia.
Topics: Dyskinesias; Facial Muscles; Female; Humans; Infant; Male; Mobius Syndrome; Retrospective Studies
PubMed: 31713842
DOI: 10.1111/dmcn.14379 -
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 -
Annual Review of Vision Science Sep 2021Abnormalities in cranial motor nerve development cause paralytic strabismus syndromes, collectively referred to as congenital cranial dysinnervation disorders, in which... (Review)
Review
Abnormalities in cranial motor nerve development cause paralytic strabismus syndromes, collectively referred to as congenital cranial dysinnervation disorders, in which patients cannot fully move their eyes. These disorders can arise through one of two mechanisms: () defective motor neuron specification, usually by loss of a transcription factor necessary for brainstem patterning, or () axon growth and guidance abnormalities of the oculomotor, trochlear, and abducens nerves. This review focuses on our current understanding of axon guidance mechanisms in the cranial motor nerves and how disease-causing mutations disrupt axon targeting. Abnormalities of axon growth and guidance are often limited to a single nerve or subdivision, even when the causative gene is ubiquitously expressed. Additionally, when one nerve is absent, its normal target muscles attract other motor neurons. Study of these disorders highlights the complexities of axon guidance and how each population of neurons uses a unique but overlapping set of axon guidance pathways.
Topics: Axons; Cranial Nerve Diseases; Eye Movements; Humans; Motor Neurons; Strabismus
PubMed: 34081534
DOI: 10.1146/annurev-vision-093019-114307 -
Neurosurgical Focus: Video Apr 2022Petroclival meningiomas are surgically challenging due to the surrounding neurovascular structures. Petroclival meningiomas located inferior to the oculomotor nerve and...
Petroclival meningiomas are surgically challenging due to the surrounding neurovascular structures. Petroclival meningiomas located inferior to the oculomotor nerve and superior or medial to the abducens nerve are ideal for an endoscopic endonasal transclival approach because this prevents the need to work across cranial nerves, limiting operative risk. The authors present a case of a 45-year-old woman with a growing petroclival meningioma that was distorting the pons. In the video they demonstrate the technique and discuss nuances of petroclival meningioma resection via an endoscopic endonasal transclival approach with posterior clinoidectomy. The video can be found here: https://stream.cadmore.media/r10.3171/2022.1.FOCVID21209.
PubMed: 36285000
DOI: 10.3171/2022.1.FOCVID21209 -
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