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Poland syndrome refers to a chest wall disorder in which there is a deficiency of the pectoral musculature. Möbius syndrome is a rare disorder in which there is absence or hypoplasia of the facial or abducens nerve, either unilaterally or bilaterally. Described here is a case in a newborn male in which both conditions manifest simultaneously as Poland-Möbius syndrome. The imaging findings here serve as a useful guide for the radiologist and ordering providers by reinforcing the need for dedicated cranial nerve imaging in patients who have deficiencies in anterior chest wall musculature. Keywords: Cranial nerve palsy, Möbius syndrome, Poland syndrome

The detection of pathological conditions related to the twelve cranial pairs represents a significant challenge for both clinicians and radiologists; imaging techniques are fundamental for the management of many patients with these conditions. In addition to knowledge about the anatomy and pathological entities that can potentially affect the cranial pairs, the imaging evaluation of patients with possible cranial pair disorders requires specific examination protocols, acquisition techniques, and image processing. This article provides a review of the most common symptoms and syndromes related with the cranial pairs that might require imaging tests, together with a brief overview of the anatomy, the most common underlying processes, and the most appropriate imaging tests for different indications.

Bilateral ophthalmic-ethmoidal dural arteriovenous fistulas (DAVFs) are extremely rare and may present as complex lesions. These DAVFs are associated with high risk of intracranial hemorrhage but can be presented with ocular symptoms, cranial nerve palsy or epistaxis. Endovascular approaches have been used to manage an increasing proportion of complex intracranial DAVFs safely and with good clinical results. We present a patient with subdural hematoma and severe epistaxis due to ruptured bilateral ophthalmic-ethmoidal DAVF that was successfully treated by transarterial embolization with precipitating hydrophobic injectable liquid. Keywords: Embolization, AV fistula, Bilateral ophthalmic, PHIL

Purpose: To describe the ophthalmic symptoms and histopathological findings in a case of primary neurolymphomatosis (NL). Observations: A man in his 60s with a prior diagnosis of chronic inflammatory demyelinating polyneuropathy developed facial numbness, diplopia, drooling, and difficulty swallowing. Over a 3-month period, he developed total ptosis and ophthalmoplegia of the right eye with a dilated, non-reactive pupil considered secondary to cranial nerve III and VI palsies. His left pupil subsequently became non-reactive to light and accommodation, and extraocular motility of the left eye was partially limited in all directions of gaze without ptosis. Autopsy findings included primary NL, diffuse large B-cell lymphoma of activated B-cell subtype, involving right and left cranial nerves V, VI, IX, and X; spinal nerve roots; both femoral nerves; and extrascleral, intrascleral, and intraocular short and long posterior ciliary nerves with extension into the adjacent choroid of both eyes. No evidence of lymphoma was identified elsewhere in the body. Conclusions and importance: Our patient is only the second histological demonstration of ciliary nerve involvement by NL, and the first, to our knowledge, of primary NL spreading secondarily from the ciliary nerves into the choroid. Our patient demonstrates that NL, though rare, should be included in the differential diagnosis of ocular cranial nerve palsies and ophthalmoplegia. Keywords: Neurolymphomatosis, Lymphoma, Cranial nerve palsy, Ophthalmoplegia, Diplopia, Ciliary nerve

Purpose To report a case of acute posterior multifocal placoid pigment epitheliopathy (APMPPE) associated with cranial nerve (CN) III palsy. Observations A 20-year-old woman developed bilateral anterior uveitis, which resolved with topical steroids. Three weeks later she exhibited posterior pole lesions in both eyes, corresponding with a diagnosis of APMPPE, as confirmed by multimodal imaging. Two days later the patient presented with right CN III palsy. The patient was started on oral prednisone, which was gradually tapered off. Signs and symptoms improved rapidly, with complete resolution within two months. Conclusion and importance Though rare, APMPPE may present with neurological involvement, as in this previously unreported association with CN III palsy. Unlike uncomplicated APMPPE cases, in patients with neurological manifestations systemic therapy is advocated.

Case report A 4 year-old girl was referred to our hospital after have suffered a severe accident. The patient was diagnosed with complete third nerve palsy in her right eye and Purtscher retinopathy in her left eye. Discussion Purtscher retinopathy is a rare condition. The diagnosis is made on clinical ground and its treatment is not well defined although it is believed that systemic steroids could improve the visual outcome. Traumatic third nerve palsy has a poor spontaneous recovery. The use of botulinum toxin might be useful in children to improve the recovery rate, maintaining binocularity, and avoiding amblyopia in other cases.

Purpose Incomplete recovery from injury to the third cranial nerve results in ocular misalignment and associated diplopia. Our aim in this study was to describe and evaluate strabismus surgery strategies aimed at restoring functional, single binocular vision in this population. Design Retrospective review. Participants We studied 12 adult patients with acquired partial third cranial nerve palsy who underwent strabismus surgery. Methods The 12 consecutive patients with residual third nerve palsy were selected from among the patients seen between 2000 and 2010 in the clinical practice of 1 strabismologist (M.F.). Complete pre- and postoperative ophthalmologic and orthoptic examinations were performed in each patient. The patients presented with isolated hypotropias (n = 7) and exohypotropias (n = 5). Strabismus surgery included: contralateral superior rectus recession, ipsilateral inferior rectus recession, vertical transposition of horizontal recti, horizontal rectus muscle surgery, or combined horizontal and vertical muscle surgery. Complete surgical success was defined as postoperative alignment within 5 prism diopters (PD) of orthotropia and the absence of diplopia in functional positions of gaze. The average follow-up was 23 months (range, 7 to 81 months). Results The mean preoperative vertical and horizontal deviations were 19 PD hypotropia (8–40 PD) and 19 PD exotropia (6–40 PD), respectively. The mean postoperative deviations were 2 PD hypotropia (0–8 PD) and 1 PD exotropia (0–6 PD). Complete surgical success was achieved in 7 of 12 patients. Partial success was attained in 5 of 12 patients, who experienced significant improvement but required postoperative use of a prism. Conclusions This study indicates that patients with incomplete third cranial nerve paralysis may enjoy good functional and cosmetic outcomes with strabismus surgery.

Resumen Objetivo Llamar la atencion sobre la existencia de la fractura del condilo occipital y la facilidad con la que pasan desapercibidas durante la atencion del paciente politraumatizado. Es una lesion poco frecuente, especialmente en pacientes adolescentes, y debe tenerse en cuenta ante un traumatismo craneal con dolor cervical por sus potenciales consecuencias si estas fracturas no se tratan correctamente. La exploracion radiografica puede parecer normal, debiendo hacer el diagnostico mediante tomografia computarizada. El tratamiento de eleccion depende de la estabilidad de la fractura. Material y metodo Dos pacientes varones de 17 y 40 anos involucrados en sendos accidentes de motocicleta, presentaron una fractura impactada del condilo occipital. En ambos casos se realizo tratamiento conservador con collar cervical rigido. Resultados Se obtuvieron buenos resultados funcionales sin secuelas neurologicas. Conclusion y relevancia clinica El conocimiento y sospecha de esta infrecuente entidad y su correcto diagnostico y tratamiento es crucial para conseguir un buen resultado funcional, para asi evitar potenciales lesiones neurologicas asociadas.

Isolated complete third cranial nerve palsy following penetrating head injury has been rarely reported. We report a very rare case of complete sudden unilateral oculomotor nerve palsy following penetrating injury and its MR imaging features. A 17-year-old man sustained clinically subtle penetrating injury to the left side of the head with instantaneous isolated oculomotor palsy on the same side. MR imaging followed by CT study revealed the path of the penetrating nail and gave evidence for the mechanism of injury of the third nerve.

Skull bone metastasis from hepatocellular carcinoma (HCC) is relatively rare, and it occasionally causes cranial nerve palsy. We describe radiographic imaging findings in two cases of HCC who developed cranial nerve palsy due to skull bone metastasis, and in whom the metastases were swiftly diagnosed and treated.

Background Vagal paragangliomas cannot be resected without sacrifice of the vagal nerve. The risk of bilateral vocal cord palsy has been reason to postpone treatment of this benign and slow growing neoplasm in hereditary cases. Postponement could be considered for solitary cases as well. Methods An institute-based review of 48 patients with vagal paragangliomas over the past 30 years was performed. Results Forty-eight patients with 58 vagal paragangliomas were studied. All but 4 patients had multiple paragangliomas and should be considered hereditary cases. The 10 patients that underwent an operation lost the vagal nerve; 60% of them had additional cranial nerve palsy postoperatively. In the group of patients who were followed for an average period of 8.5 years, 3 patients (8%) developed cranial nerve palsy. Conclusions Aggressive treatment of vagal paragangliomas leads to unnecessary early loss of vagal nerve function. A period of clinical and radiologic follow-up preceding an operation may lead to prolonged preservation of voice and swallowing functions in these patients, without grave consequences for other lower cranial nerves.

Idiopathic hypertrophic cranial pachymeningitis (IHCP) is a rare inflammatory disease which is sometimes difficult to diagnose and can lead to misinterpretations of the clinical and imaging findings. The main clinical manifestations are headache, ataxia and cranial nerve palsy. In most of the reported patients continuous medication is needed to avoid disease recurrence. We present a female patient with an 8-year follow-up, no clinical regression and no need for any further medical treatment. Even though most patients with IHCP experience recurrence after diagnosis and initial treatment there were no clinical or imaging signs of relapse in our patient. Our patient is still not under any medical or surgical treatment due to the lack of any significant symptoms.

Brainstem alone is involved very infrequently in patients with neurocysticercosis; usually, it occurs in association with disseminated form of the disease. Isolated involvement of the third nerve is commonly due to vascular causes. We are reporting a case due to cysticercus lesion presenting as isolated third-nerve involvement with sparing of the pupil. The diagnosis of neurocysticercosis was established by the presence of characteristic granulomatous lesions in the midbrain along with positive ELISA for cysticercal antigen in the cerebrospinal fluid. The patient responded well to corticosteroids with almost complete recovery. A follow-up MRI scan showed a significant decrease in the size of the lesion.

Resume Le syndrome de Collet-Sicard est une affection rare, defini par une paralysie unilaterale des quatre derniers nerfs crâniens (IX a XII). Il se distingue du syndrome de Villaret par l’absence d’atteinte sympathique associee. Les etiologies classiques du syndrome de Collet-Sicard comportent les tumeurs de la base du crâne, les traumatismes et les atteintes vasculaires. Nous rapportons le cas d’un patient de 57 ans ayant presente un syndrome de Collet-Sicard gauche lie a une dissection carotidienne dans les suites d’un traumatisme cervical mineur.

BACKGROUND AND OBJECTIVE: To characterize the clinical features, etiology and prognosis of pontine hemorrhages (PH). PATIENTS AND METHOD: Descriptive study of 14 patients with PH included in the Sagrat Cor Hospital of Barcelona Stroke Registry over a 19-year period. The vascular risk factors and clinical profiles in PH and subcortical hemorrhage group (n = 133) were compared. RESULTS: PH accounted for 0.36% of all acute strokes (n = 3,808) and 3.4% of all intracerebral hemorrhages. Hypertension was present in 78.6%, diabetes mellitus in 21.4% and hyperlipidemia in 21.4%. Pure motor hemiparesis was present in 2 patients (14.3%). The overall in-hospital mortality rate was 50%. Dizziness (odds ratio = 27.38; 95% confidence interval, 2.43-218.7) and cranial nerve palsy (odds ratio = 26.28; 95% confidence interval, 5.84-118.17) were independent predictors related to PH. CONCLUSIONS: Hemorrhagic pontine stroke is infrequent, presents a high in-hospital mortality and clinical profiles were identified in PH versus subcortical hemorrhages.

We describe the locked-in syndrome in a 31-yr-old patient after right upper lobectomy for suspected metastasis. After surgery, vertical eye movement was her only means of communication. She remained in a 'locked-in' state for 7 months before dying. Post-mortem examination showed extensive metastatic tumour deposition in the ventral pons. We discuss the clinical features of this syndrome and factors that may affect onset, diagnosis and management.

Villaret's syndrome is an extremely uncommon condition in which the four lower cranial nerves and the cervical sympathetic are selectively affected. Most cases described thus far have been attributed to either trauma or space-occupying lesions in the posterior retroparotid space. This report demonstrates a first case of "pure" Villaret's syndrome caused by jugular vein thrombosis secondary to previous mastoiditis.

Data source: • Retrospective chart review • All patients undergoing surgery for complete CN 6 (CN6) palsy by two surgeons (MDM and SA) at Kellogg Eye Center from January 1, 1985 and August 1, 2014. Inclusion criteria: • Complete CN6 palsy • Any etiology • Age between 6 months and 99 years • Managed with full tendon vertical rectus muscle transpositions with Foster-Augmentation or resection of vertical rectus muscles prior to transposition Exclusion criteria (Figure 1): • Graves eye disease • Prior transposition procedure on involved eye • Bilateral transpositions • Partial CN6 palsy • Brain stem depression • Staged procedure or simultaneous surgery on other extraocular muscles

A number of focal neurological conditions have been described at high altitude. This report presents two cases of isolated unilateral lateral rectus palsies developing shortly after ascent to high altitude. In both cases concomitant altitude illness was not present and symptoms resolved completely within weeks to months. Possible etiologies are discussed.

Isolated involvement of the 5th through 10th cranial nerves, in blunt head trauma, without any other neurological deficit, is extremely rare. Two such cases are reported and relevant literature reviewed. The mechanics of trauma involved and the poor recovery is outlined.

Cranial nerve palsy is a rare complication of dental extraction. This article presents a report of an incomplete seventh nerve paralysis and branch third nerve palsy occurring during extraction of a mandibular third molar. Diagnostic and treatment considerations are discussed, including a review of pertinent neuroanatomy.

Fractures of the sella turcica are rare. Recognition of fractures in this area is very important as serious immediate and delayed neurological, endocrine and vascular complications may occur. Most of these patients will develop at least one cranial nerve palsy; a significant number will develop hypopituitarism and a few will develop local vascular abnormalities.