Isolated dysarthria due to hypoglossal palsy caused by supratentorial ischemic stroke: Radiological and electropsyological analysis.

OBJECTIVES: Isolated dysarthria caused by stroke is a rare condition and generally seen in infratentorial lesions, especially in cerebellar lesions. Isolated dysarthria associated with supratentorial ischemic lesions are also very rare and, these conditions were shown in only a few cases. In this study, six patients characterized by IHP due to supratentorial ischemic lesions are presented. PATIENTS AND METHODS: Physical examinations of patients were done by two different neurologists. The risk factors for stroke were determined. Localisations of lesions were identified by using magnetic resonance images(MRI). Corticolingual tract affection without sensorial and other corticospinal tract involvement was confirmed using Somatosensory evoked potentials(SEPs) and Transcranial Magnetic Stimulation(TMS). RESULTS: Unilateral lingual paresis was detected in all of the patients. The lesions were demonstrated on MRI slices. All lesions were on the same localization. The affection of the corticolingual tract without any other motor and sensory tract involvement was proven electrophysiologically. CONCLUSION: Corticospinal and corticobulbar fibers are very close to each other. Therefore common involvement is expected in cerebrovascular diseases. However, six patients with IHP caused by supratentorial ischemic lesions were reported in this study. Thus, a possible map of the corticolingual tract was drawn.

Burkitt lymphoma presenting with fever of unknown origin and isolated hypoglossal nerve palsy.

Fever of unknown origin (FUO) continues to present a clinical conundrum for even expert practitioners. The syndrome of FUO has over 200 possible etiologies. Burkitt lymphoma (BL) is a highly aggressive B-cell non-Hodgkin lymphoma with only 1,200 US adult cases reported annually. Fever, night sweats, and weight loss, otherwise known as B symptoms, are common early symptoms of BL. Nerve palsy, especially isolated hypoglossal nerve palsy (IHNP), is rarely seen as a presenting sign in any pathological condition. A case report of FUO and IHNP as the presenting manifestations of BL is presented. The rarity of IHNP and its clinical features delayed the recognition of this syndrome and emphasizes the value of a thorough understanding of the physical examination and the association of unusual clinical findings with a readily identifiable clinical syndrome.

Hypoglossal Nerve Palsy due to Compression by a Persistent Primitive Hypoglossal Artery: Case Report.

A 51-year-old Japanese woman was admitted to our hospital because of speech difficulty following severe headache. Neurological examination showed dysarthria and tongue weakness on the right side, indicating right hypoglossal nerve palsy. Needle electromyography of the right side of the tongue showed fibrillation potentials. Magnetic resonance angiography and computed tomography angiography revealed a right, persistent, primitive hypoglossal artery (PPHA) that met Lie's diagnostic criteria. Digital subtraction angiography showed an extended PPHA with irregular caliber in the portion running through the right hypoglossal canal. We diagnosed compression neuropathy of the hypoglossal nerve due to PPHA enlargement based on the findings of ipsilateral hypoglossal nerve palsy, fibrillation that indicated peripheral nerve palsy, and the enlarged diameter of the portion of the PPHA running through the right hypoglossal canal. We prescribed antihypertensive therapy. At 1 year after onset, her tongue weakness was alleviated. Clinicians should consider compression neuropathy due to a PPHA as one of the possibilities in the differential diagnosis of hypoglossal nerve palsy.

Case of idiopathic isolated unilateral hypoglossal nerve palsy with spontaneous recovery.

Isolated hypoglossal nerve palsy is uncommon because of its intimate relationship with the other lower cranial nerves. Keane reported that tumours, predominantly malignant, were the most common cause of hypoglossal nerve palsy. We report a case of isolated idiopathic unilateral hypoglossal nerve palsy in a 45-year-old Caucasian male where no cause could be identified despite extensive investigations. There was uncertainty around prognosis at onset due to the rarity of this condition. In the absence of a cause, an early referral to the speech and language therapist was made and interestingly our patient made an almost complete recovery within 18 months of onset. In a small case series, it has been reported that though rare, idiopathic hypoglossal nerve palsy has an excellent outcome in most cases, similar to the more common idiopathic seventh cranial nerve palsy (Bell's Palsy).We recommend an early referral for physiotherapy in such cases.

Cervical Vestibular Evoked Myogenic Potential in Hypoglossal Nerve Schwannoma: A Case Report.

BACKGROUND: Schwannoma of the hypoglossal nerve is rare. This case report documents an atypical abnormality of the cervical vestibular evoked myogenic potential (cVEMP) in a patient with schwannoma of the hypoglossal nerve. The observed abnormality was attributed to the proximity of the hypoglossal nerve to the spinal accessory nerve in the medullary cistern and base of the skull. PURPOSE: To report cVEMP abnormality in a patient with hypoglossal nerve schwannoma and provide an anatomical correlation for this abnormality. RESEARCH DESIGN: Case report. STUDY SAMPLE: A 44-yr-old woman. DATA COLLECTION: Pure-tone and speech audiometry, tympanometry, acoustic stapedial reflex, auditory brainstem response, and cVEMP testing were performed. RESULTS: The audiological test results were normal except for the absence of cVEMP on the lesion side (right). CONCLUSIONS: A cVEMP abnormality indicating a compromised spinal accessory nerve was observed in a patient with hypoglossal nerve schwannoma. This case report highlights the importance of recording cVEMP in relevant neurological conditions and provides clinical proof for the involvement of the spinal accessory nerve in the vestibulocollic reflex pathway.

Isolated Hypoglossal Paralysis Caused by Ischemic Infarction in the Centrum Semiovale.

In the present report, we discuss the case of a 66-year-old woman with isolated unilateral hypoglossal paralysis due to cerebral infarction in the centrum semiovale. To date, it has hardly been discussed where the corticolingual tract passes through in the centrum semiovale. Brain magnetic resonance imaging revealed a small ischemic infarction in the contralateral centrum semiovale. We could demonstrate a route of the corticolingual tract.

[A Case of Hypoglossal Nerve Palsy after Cervical Spine Surgery].

A 46-year-old woman with cervical disc herniation underwent C4-6 laminoplasty and C4-5 foraminotomy under general anesthesia. The patient complained of the tongue deviation toward the right after surgery. She underwent several examinations and was diag- nosed as right hypoglossal nerve palsy caused by peripheral nerve disorder. It was considered that the main damage was the external branch of hypoglossal nerve which was likely to occur as a complication of compression because of the location. The cause of hypoglossal nerve palsy was assumed to be possibly direct compression of the hypoglossal nerve by the tracheal tube. In prone position surgery, it is important to take care of pressure to the tongue because the intraoral space tends to be reduced by edema of the face and neck.

Progressive cranial nerve involvement and grading of facial paralysis in gelsolin amyloidosis.

INTRODUCTION: Hereditary gelsolin amyloidosis (GA) is a rare condition caused by the gelsolin gene mutation. The diagnostic triad includes corneal lattice dystrophy (type 2), progressive bilateral facial paralysis, and cutis laxa. Detailed information on facial paralysis in GA and the extent of cranial nerve injury is lacking. METHODS: 29 GA patients undergoing facial corrective surgery were interviewed, examined, and studied electroneurophysiologically. RESULTS: All showed dysfunction of facial (VII) and trigeminal (V) nerves, two-thirds of oculomotor (III) and hypoglossal (XII) nerves, and half of vestibulocochlear (acoustic) (VIII) nerve. Clinical involvement of frontal, zygomatic, and buccal facial nerve branches was seen in 97%, 83%, and 52% of patients, respectively. Electromyography showed marked motor unit potential loss in facial musculature. CONCLUSIONS: Cranial nerve involvement in GA is more widespread than previously described, and correlates with age, severity of facial paralysis, and electromyographic findings. We describe a grading method for bilateral facial paralysis in GA, which is essential for evaluation of disease progression and the need for treatment.

Clival osteomyelitis and hypoglossal nerve palsy--rare complications of Lemierre's syndrome.

An increasingly reported entity, Lemierre's syndrome classically presents with a recent oropharyngeal infection, internal jugular vein thrombosis and the presence of anaerobic organisms such as Fusobacterium necrophorum. The authors report a normally fit and well 17-year-old boy who presented with severe sepsis following a 5-day history of a sore throat, myalgia and neck stiffness requiring intensive care admission. Blood cultures grew F. necrophorum and radiological investigations demonstrated left internal jugular vein, cavernous sinus and sigmoid sinus thrombus, left vertebral artery dissection and thrombus within the left internal carotid artery. Imaging also revealed two areas of acute ischaemia in the brain, consistent with septic emboli, skull base (clival) osteomyelitis and an extensive epidural abscess. The patient improved on meropenem and metronidazole and was warfarinised for his cavernous sinus thrombosis. He has an on-going left-sided hypoglossal (XIIth) nerve palsy.

Occipital Condyle Syndrome as an Initial Presentation of Lung Cancer: A Case Report.

PURPOSE: Occipital condyle syndrome (OCS) is a rare cause of headache. This study herein reports a case in which a unique headache and tongue deviation appear as symptoms of the first presentation of a malignant tumor. CASE REPORT: A healthy 67-year-old male presented with a unilateral shooting pain in the occipital region, accompanied by slurred speech and difficulty swallowing. Neurological examinations later revealed atrophy and mild fasciculation of the tongue. The clinical symptoms and MRI results suggested OCS. Screening for tumor markers showed an elevated CEA. The chest CT revealed a lobulated soft-tissue mass in the lower left lobe, and a CTguided biopsy confirmed the diagnosis of adenocarcinoma. A whole body bone scan found multiple foci. The adenocarcinoma was graded pT2bN3M1b, stage IV. The headache improved with a prescription of prednisone, 60 mg to be taken daily. With three months of treatment, clinical examinations showed that the patient was free of pain and that there had been no progression of the atrophy or deviation of the tongue. CONCLUSION: The possible etiology of OCS includes a primary tumor or metastatic lesion that directly invades the base of the skull. Determining the underlying causes of OCS can be challenging, but MR imaging is currently the diagnostic tool of choice. An awareness of the features of OCS in healthy adults may be able to lead to earlier diagnosis of the underlying etiology and efficient relief of the symptoms.