Cranial Neuralgias.

OBJECTIVE: The cranial neuralgias are relatively rare, but recognizing these syndromes and distinguishing among them is critical to reducing unnecessary pain and disability for affected patients. Despite their distinctive features, cranial neuralgias may go undiagnosed or misdiagnosed for several years. A notable proportion of cranial neuralgia presentations are due to secondary causes and require targeted treatment. The purpose of this article is to review the diagnosis and management of cranial neuralgias encountered in clinical practice. LATEST DEVELOPMENTS: In 2020, the International Classification of Orofacial Pain was released for the first time. Modeled after the International Classification of Headache Disorders, it includes updated terminology for cranial neuralgias. The underlying pathophysiology of the cranial neuralgias is currently believed to be rooted in both peripheral and central nociceptive systems. In addition, a growing number of familial cases are being identified. Recent therapeutic advancements include a better understanding of how to utilize older therapies and procedures more effectively as well as the development of newer approaches. ESSENTIAL POINTS: Cranial neuralgia syndromes are rare but important to recognize due to their debilitating nature and greater likelihood of having potentially treatable underlying causes. While management options have remained somewhat limited, scientific inquiry is continually advancing the understanding of these syndromes and how best to address them.

Imaging of Cranial Neuralgias.

Cranial neuralgia (CN) can cause significant debilitating pain within a nerve dermatome. Accurate diagnosis requires detailed clinical history and examination, understanding of pathophysiology and appropriate neuroimaging to develop an optimal treatment plan. The objective of this article is to review and discuss some of the more common CNs including trigeminal neuralgia and its associated painful neuropathies, occipital neuralgia, and less common glossopharyngeal neuralgia (GPN). The neuroanatomy, pathophysiology, diagnostic imaging, and treatment of each of these pathologies are reviewed with emphasis on the role of CT and MR imaging findings in guiding diagnosis. Although CT is often used to initially identify an underlying cause such as neoplasm, infection, or vascular malformation, MRI is optimal. Clinical history and examination findings along with MRI constructive interference steady state/fast imaging employing steady-state acquisition sequences and MRA of the brain can be used to distinguish between primary and secondary cranial neuropathies and to discern the best treatment option. Pharmacologic and noninvasive therapy is the first-line of treatment of these cranial and cervical neuralgias. If symptoms persist, stereotactic radiosurgery is an option for some patients, although microvascular decompression surgery is the most curative option for both trigeminal and GPN. Refractory occipital neuralgia can be treated with a nerve block, an ablative procedure such as neurectomy or ganglionectomy, or more recently occipital nerve stimulation.

Middle Meningeal Artery Embolization for Chronic Subdural Hematoma Using N-Butyl Cyanoacrylate With D5W Push Technique.

BACKGROUND: Middle meningeal artery (MMA) embolization has been recognized as a promising treatment for patients with subdural hematoma (SDH). OBJECTIVE: To present the technical feasibility and efficacy of n-butyl cyanoacrylate (n-BCA) embolization in the largest consecutive cohort to date. METHODS: We retrospectively reviewed our consecutive cases of recurrent SDH treated with MMA embolization using diluted n-BCA with the "sugar rush" technique. In brief, a 2.1-Fr microcatheter was used to selectively catheterize the frontal and posterior branches of the MMA. 5% dextrose in water (D5W) was injected through an intermediate catheter while injecting n-BCA through the microcatheter. Complete obliteration of MMA and lack of SDH recurrence in a 3-6 months follow-up computed tomography scan were defined as efficacy outcomes. Cranial nerve palsy, vision loss, transient neurological deficit, and stroke were defined as safety outcomes. RESULTS: A total of 61 patients were identified with a mean (±standard deviation) age of 62.5 ± 9 years. In 6 patients (10%), coil embolization of the origin of the frontal or posterior branch was performed because super-selective catheterization of the branch was unsuccessful because of tortuous anatomy. Complete obliteration of frontal and posterior branches was achieved in 100% of the cases. Recurrent SDH was seen in 3 patients (5%). No incidence of cranial nerve palsy, vision loss, or stroke occurred. One patient suffered a transient neurological deficit. CONCLUSION: MMA embolization using diluted n-BCA with concomitant D5W injection is associated with a high degree of distal penetration and complete branch occlusion and minimal risk of cranial nerve palsy or other thromboembolic complications.

[Skull base metastases with cranial nerve deficits : Clinical profile of a severe disease]. / Schädelbasismetastasen mit Hirnnervenaffektionen : Klinischer Steckbrief einer schwerwiegenden Erkrankung.

BACKGROUND AND PURPOSE: Skull base metastases are a severe complication of various malignant tumors. If cranial nerves are involved even small lesions can cause significant symptoms. Specific clinical characteristics like neurological symptoms, associated primary tumors, prognosis and optimal treatment are poorly defined and are systematically described in this article. METHODS: In a monocentric retrospective study patients with skull base metastases and cranial nerve deficits who received treatment between 2006 and 2018 were analyzed concerning clinical characteristics at initial diagnosis, treatment and course of the disease. RESULTS: In this study 45 patients with skull base metastases and cranial nerve deficits were included. The most frequent primary tumors were prostate cancer (27%), breast cancer (22%) and multiple myeloma (16%). The most involved cranial nerves were trigeminal nerve (42%), oculomomotor nerve (33%) and facial nerve (27%). Of the patients 84% had additional bone metastases outside the skull base. Dural infiltration or meningeal carcinomatosis were each observed in 13% of the patients. After radiotherapy cranial nerve deficits remained stable in 61% of all cases and in 22% symptoms improved. Median overall survival from treatment was 8 months (range 0.4-51 months). Patients with dose-escalated radiotherapy appeared to live longer (16.4 months vs. 4.7 months). This effect persisted in a multivariate analysis including the Karnofsky index, number of metastases, primary tumor and radiation dose (HR 0.37, p = 0.02). CONCLUSION: Skull base metastases with cranial nerve deficits are complex diseases with poor prognosis. Precise diagnosis and treatment are required. Further research is needed to improve treatment.

[Patient treated for severe Covid-19 with paralysis of cranial nerves]. / Kranialnervspareser uppstod hos Iva-vårdad covid-19-patient.

Patients with Covid-19 can experience neurological complications, for example cranial nerve palsy. We present a case with a patient treated for severe Covid-19 infection. She was intubated for 16 days and was ventilated in the prone-position for several hours a day during her care in the intensive care unit (ICU). She developed paralysis of the left facial nerve, observed while intubated. After extubation the patient was hoarse and had dysphagia and examination showed paralysis of the left hypoglossal nerve with the tongue deviating to the left and of the left vagus nerve causing a paralysis of the left vocal cord. It is impossible to know whether the paralysis of the three cranial nerves was due to direct damage by the Covid-19 virus or due to compression of the nerves during the ICU care. As facial nerve palsy has been shown to be more common in patients with Covid-19, we believe that the paralysis in our patient was due to a combination of both.

The Diagnosis and Management of Posttraumatic Headache with Associated Painful Cranial Neuralgias: a Review and Case Series.

BACKGROUND: Cranial neuralgias are common in the setting of posttraumatic headache. They may exacerbate underlying primary headache disorders and therefore may be overlooked in clinical practice. Frequently, cranial neuralgias generate neuropathic symptoms such as lancinating pain and sensory dysesthesias. Cranial neuralgias are identified based on a clinical history of focal neuropathic pain and physical exam findings including tenderness with palpation and percussion, at times eliciting radiating pain or paresthesias in the corresponding sensory nerve distribution. PURPOSE OF REVIEW: This article is a brief review of the literature and a retrospective report of 2 cases of posttraumatic headache with associated painful cranial neuralgias. RECENT FINDINGS: Two patients presented with headaches that met criteria for posttraumatic headache, but their history and physical examination suggested the presence of a focal painful cranial neuralgia. One patient was diagnosed with auriculotemporal neuralgia, which was exquisitely responsive to an auriculotemporal nerve block. The second patient was diagnosed with supratrochlear neuralgia, which was effectively treated with a supratrochlear nerve block. In both cases, adequate treatment of the painful cranial neuralgia resulted in significant improvement of the baseline PTH. Painful cranial neuralgias frequently occur within the clinical spectrum of posttraumatic headache, but are often undiagnosed. Treatment options for painful cranial neuralgias are often different than those traditionally employed for posttraumatic headache without cranial neuralgias, which can include peripheral nerve blockade, neuropathic medications, and in refractory cases, peripheral nerve decompression surgery.

[A case of Fisher syndrome presented by rapidly progressing bilateral palatoplegia after cytomegalovirus infection].

A 35-year-old male developed sensory abnormality of peripheral limbs and oral cavity after prior infection with diarrhea and cold symptoms. Hyperrhinolalia, nasopharyngeal reflux, double vision, and wobbling in walking rapidly progressed. Neurological examination revealed palatoplegia, omnidirectional ophthalmoplegia, hyperreflexia, sensory disturbance of extremities, and truncal and limb ataxia due to decreased deep sensation. A peripheral nerve conduction study found a slight decrease in sensory nerve action potential of the median nerve and a decrease in F wave frequency of the median nerve. Serum IgM-CMV antibody was positive on admission. After IVIg therapy, palatoplegia and ataxia markedly improved. In this case, GalNAc-GD1a and GM2 antibodies, which are often detected after CMV infection, were positive in addition to the GT1a and GQ1b antibodies, and it was assumed that these findings were associated with the palatoplegia, which is included in cranial nerve palsy. Pathophysiologically, the present case is considered to be an overlap with acute oropharyngeal palsy (AOP), which is a rare subtype of Guillain-Barre syndrome, and Fisher syndrome (FS). The clinical aspects of the present case suggest a continuous spectrum between AOP and FS.

Delayed-Onset Cranial Nerve Palsy After Transvenous Embolization of Indirect Carotid Cavernous Fistulas.

BACKGROUND: Carotid cavernous fistulas (CCF) often present with diplopia secondary to cranial nerve palsy (CNP). Immediate development of postoperative CNP has been described in the literature. This study described delayed-onset of CNP after complete and reconfirmed obliteration of the CCF and resolution of initial CNP. METHODS: A retrospective analysis was performed on patients with indirect CCF between 1987 and 2006 at 4 academic endovascular centers. Details of the endovascular procedures, embolic agents used, and complications were studied. Partial or complete obliteration was determined. Immediate and delayed cranial nerve palsies were independently assessed. RESULTS: A total of 267 patients with symptomatic indirect CCF underwent transvenous endovascular treatment. Four patients (1.5%) developed delayed abducens nerve (VI) palsy after complete resolution of presenting symptoms after embolization. Delayed presentation ranged between 3 and 13 months after complete resolution of initial double vision and cranial nerve palsies. Transvenous coil embolization through the inferior petrosal sinus was performed in all 4 affected patients. All had follow-up angiography confirming durable closure of their CCF. MRI did not show new mass lesions or abnormal soft tissue enhancement. In all 4 patients, their abducens nerve (VI) palsy remained. CONCLUSIONS: Delayed CNP can develop despite complete endovascular obliteration of the CCF. The cause of delayed CNP is not yet determined, but may represent fibrosis and ischemia. Long-term follow-up is needed even after complete neurological and radiological recovery is attained in the immediate perioperative period.

[End-of-Life Medical Care for Cranial Nerve Diseases from Ethical and Legal Perspective].

After showing the basic knowledge of law, I examined the relation ship between law and ethics. Subsequently, I explained a related law about the end-of-life stage and neurological intractable diseases based on specific cases. I analyzed the structure of the guidelines for end-of-life and dementia guidelines, which have great significance in Japan. In addition, the issue of notification (informed consent) and removal of the ventilator were examined.

Eight-and-a-half syndrome: video evidence and updated literature review.

The eight-and-a-half syndrome (EHS)-defined by the combination of a seventh cranial nerve palsy and an ipsilateral one-and-a-half syndrome-is a rare brainstem syndrome, which localises to the caudal tegmental region of the pons. We present a case of the EHS secondary to an inflammatory lesion on a previously healthy 26-year-old woman, with a literature review emphasising the relevance of aetiological assessment.

Flow diversion for internal carotid artery aneurysms: Impact of complex aneurysm features and overview of outcome.

OBJECTIVE: Flow diversion is a popular technique used to treat ordinary small, as well as complex internal carotid artery (ICA) aneurysms. We describe aneurysm occlusion rates, complications and patient outcomes in patients with ICA aneurysms treated with flow diverter stents. PATIENTS AND METHODS: We identified all consecutive patients with ICA aneurysms that were treated with flow diverter stents between 2014 and 2019 at our institution. We divided the aneurysms into two anatomic subgroups (cavernous and supraclinoid segments). All the imaging studies and medical records were reviewed for relevant features in relation to aneurysms, complications and patient outcomes. RESULTS: A total of 62 patients with 76 ICA aneurysms (72 unruptured; 4 ruptured) were treated with 70 flow diversion procedures, including six re-treatments. Complete aneurysm occlusion was achieved in 61 % of patients at 6-month follow-up (cavernous 69 %, supraclinoid 58 %), and in 69 % at the latest follow-up (mean of 18 months). Postprocedural intracranial ischemia or hemorrhage was seen in 24 % of patients, including two aneurysm ruptures after flow diversion, and related major long-term functional decline or mortality was seen in 6% of patients. Preprocedural dysfunction of extraocular muscles or vision disturbance improved moderately at best (40-60 %). At the latest follow-up, 54 patients (87 %) were functionally independent (mRS ≤2). CONCLUSION: Flow diversion for cavernous and supraclinoid ICA aneurysms demonstrated acceptable results, but complex aneurysm features cause uncertainty in predicting aneurysm occlusion probability. Patients should be informed of the non-negligible rate of complications, and of only moderate improvement rate of cranial nerve dysfunctions.

Onyx embolization of skull base paragangliomas: a single-center experience.

BACKGROUND: Skull base paragangliomas are highly vascular tumors that are often embolized before surgical resection; however, the safety and efficacy of preoperative embolization using an ethylene vinyl alcohol copolymer (Onyx; Medtronic, Dublin, Republic of Ireland) in these tumors is unknown. This retrospective cohort study evaluated patient outcomes after preoperative embolization of skull base paragangliomas using Onyx. METHODS: We retrospectively analyzed data from all patients with skull base paragangliomas who underwent preoperative Onyx embolization at our institution (January 01, 2005-December 31, 2017). Patient, tumor, embolization, and outcomes data were extracted by reviewing inpatient and outpatient clinical and imaging records. RESULTS: Seven patients were studied (5/7 [71%] female), 6 with glomus jugulares and 1 with a glomus vagale. The median age was 52 years, and the most common presenting symptom was cranial neuropathy (6/7 [86%]). The tumor vascular supply was from the ascending pharyngeal artery in all 7 cases (100%) with additional feeders including the occipital artery in 5 (71%); internal carotid artery in 3 (43%); middle meningeal, vertebral, and internal maxillary artery each in 2 (29%); and posterior auricular artery in 1 (14%). The median postembolization tumor devascularization was 80% (range, 64-95%). The only postembolization complication was a facial palsy in 1 patient. CONCLUSION: Preoperative embolization with Onyx affords excellent devascularization for the majority of skull base paragangliomas, and it may facilitate resection of these hypervascular lesions. The advantages provided by Onyx with respect to penetration of intratumoral vessels must be weighed against the risk of cranial neuropathy.

Visual dysfunction in multiple sclerosis. / Synsproblemer ved multippel sklerose.

Multiple sclerosis can give rise to signs and symptoms from the entire nervous system, including visual impairments. Visual impairments often go unreported because they are not obvious to patients, which means that doctors must ask about them specifically. Regular monitoring of vision is important, however, to provide personalised rehabilitation and assistive technologies, and thereby improve patients' functioning and quality of life.

Use of Tachosil® as an adjuvant therapeutic option in neurotrophic keratopathy in order to prevent perforation.

We present two cases of women who suffered from neurotrophic keratopathy (one of them had undergone penetrating keratoplasty) which had led to corneal thinning. Tachosil® was used as an adjuvant treatment after topical medication by itself failed in both cases. To our knowledge, there are no reported cases of the use of Tachosil® in corneal grafts.

Hypothyroidism manifesting as multiple cranial neuropathies: a case report.

INTRODUCTION: The clinical picture of hypothyroidism, including neurological symptoms, can be multiform, which may delay or hamper the correct diagnosis. CASE PRESENTATION: We present an uncommon clinical presentation of a 38-year-old Caucasian man with mild facial palsy on the left side, uvular deviation to the left with preserved gag reflex, tongue deviation to the left, lingual dysarthria, and xerosis by severe hypothyroidism. Blood tests on admission showed elevated serum creatinine of 151 µmol/L (glomerular filtration rate 47 ml/min/1.7 CKD-EPI [Chronic Kidney Disease Epidemiology Collaboration equation]), increased creatinine phosphokinase activity (1243 U/L), markedly elevated thyroid-stimulating hormone (292.2 mIU/L), low free thyroxine level (1.1 pmol/L), and free triiodothyronine level below the limit of detection (< 0.4 pmol/L). Results of brain magnetic resonance imaging and renal ultrasound were unremarkable. Lumbar puncture revealed a normal cell count in cerebrospinal fluid, with an increased protein level of 758 mg/L and a cerebrospinal fluid/serum albumin ratio of 10.5 × 10- 3/L (reference range < 6.7). Further diagnostic workup did not reveal any inflammatory or infectious systemic pathologies as an underlying cause. The patient's neurological symptoms, as well as laboratory findings including renal function, creatinine phosphokinase, and initially altered blood lipid levels, normalized with levothyroxine substitution. CONCLUSIONS: Multiple cranial neuropathy is an uncommon clinical finding in hypothyroidism, which is an important differential diagnosis in the workup of new neurological deficits.

Delayed diagnosis of odontoid peg osteomyelitis with bilateral X and XII cranial nerve palsies.

Upper cervical osteomyelitis is rare. Its presenting features are fever and neck pain, but rarely it can involve lower nerves. MRI is the main imaging modality, but it is difficult to interpret due to the unique anatomy of C1 and C2 vertebra and complex intervertebral joint. We describe a case of a 67-year-old woman, who presented with the complaint of loss of voice, neck pain and fever for 5 days. Despite repeated imaging of neck, the diagnosis was not reached. As the patient's condition continued to deteriorate, clinical signs of bilateral 10th and 12th cranial nerve paralysis appeared and lead to a focused workup for base of skull pathology. Discussion with the radiologist helped guide the imaging protocol, which leads to the correct diagnosis being made. Treatment was tailored by blood cultures and available images. Temporary immobilisation with a cervical collar and a total of 12 weeks of antibiotics lead to complete remission.