Bilateral Seventh Nerve Palsy Research Articles

EP 75. Rare cause of bilateral seventh nerve palsy – Importance of optic nerve sonography before lumbar puncture

A 23 year old female patient with normal body mass index was admitted because of right sided facial weakness. At least for 3 years she suffered continous headache bifrontal which required daily use of analgetics. Vision was described as blurred for minutes on a few days per month, no other symptoms were stated. Medical examination showed a bilateral seventh nerve palsy, symptoms in the left face were slight. There were no signs of inflammation neither on laboratory values nor clinically. Optic nerve sonography showed an elevated optic nerve sheath diameter and an elevated optical disc bilaterally. CSF opening pressure was elevated significantly (35 cm H 2 O). CSF analysis was normal concerning protein level and cell count. Thrombosis of cerebral veins and sinus was excluded by means of MRI. Despite a removal of only 5 ml CSF, facial palsy improved completely and headache was much better for some days. We diagnosed a idiopathic intracranial hypertension. Acetazolamide was prescribed but it was taken only for some days because of adverse effects. After 6 weeks she got a second CSF removal of 25 ml. The CSF opening pressure was 46 cm H 2 O. Optic nerve sonography was unchanged with papilledema. Since 8 month until now, there was no recurrent headache and no facial palsy. The findings of the optic nerve sonography lead us to take the CSF opening pressure which has influenced the clinical management. This resulted in a complete recovery of a long lasting headache history. Fig. 1 . Elevation of the optical disc (papilledema).

One-and-a-Half Syndrome With Facial Diplegia: The 15 1/2 Syndrome?

We recently examined a 67-year-old man who demonstrated findings consistent with a unilateral one-and-a-half syndrome and a bilateral lower motor neuron facial palsy (1 1/2 + 7 + 7 = 15 1/2). The patient had the sudden onset of dizziness, nausea, and blurring of vision. He smoked heavily and had a history of diabetes mellitus. Examination disclosed a complete right horizontal conjugate gaze palsy and a right adduction deficit. Oculocephalic stimulation did not improve the defective eye movements. Convergence was relatively spared. Vertical ocular movements and alignment were normal and ptosis was absent. The pupils were equal in size and normally reactive to light and a near stimulus. The remainder of the neurologic examination was normal. One day later, a bilateral lower motor neuron facial palsy abruptly developed, including bilateral flattening of the nasolabial fold and absent forehead wrinkling. Brain computed tomography was normal. FLAIR and T2-weighted brain magnetic resonance imaging disclosed high signal in the lower pontine tegmentum, findings considered typical of stroke (Fig. 1). A facial nerve conduction and blink reflex study confirmed a bilateral lower motor neuron facial palsy.FIG. 1: Axial FLAIR (A), axial T2-weighted (B), sagittal T2- weighted (C), and diffusion-weighted (D) magnetic resonance images show increased signal (arrow) consistent with ischemic stroke in the paramedian dorsal pontine tegmentum bilaterally.When last examined 2 months after the onset of symptoms, the patient showed only a minimal deficit in right horizontal gaze. Horizontal gaze palsy in one-and-a-half syndrome may result from lesions in the abducens nucleus, in the abducens nerve and the medial longitudinal fasciculus, or in the pontine paramedian reticular formation (PPRF) (1,2). Isolated PPRF lesions are associated with retained vestibulo-ocular movement. If the vestibulo-ocular movement is abolished, the lesion lies rostral or caudal to the PPRF (5,6). Because our patient showed limitation of oculocephalic abduction toward the right side, we presume that the lesion lies in the abducens nucleus or fascicle rather than in the PPRF (2-7). More rapid resolution of ipsilateral adduction compared with abduction favors involvement of the abducens fascicle rather than the nucleus. An alternative hypothesis is that these findings reflect the relative vulnerability of abducens motor neurons innervating the lateral rectus (2). The one-and-a-half syndrome is rarely an isolated finding (7), being usually associated with cranial nerve palsies (facial in 75%), hemiplegia (30%), or hemihypesthesia (35%) (3,4). A lower motor neuron facial nerve palsy is the most commonly associated finding, resulting from involvement of the genu of the intraaxial fasciculus of the facial nerve in the pontine tegmentum (2,4). Although the combination of a one-a-a-half syndrome and an ipsilateral seventh nerve palsy has been described ("eight-and-a-half syndrome" (2)), we are unaware of a previous description of a one-and-a-half syndrome with bilateral seventh nerve palsies, as our patient had. We attribute these findings to a bilateral tegmental pontine lesion involving the right abducens nucleus or fasciculus, bilateral or right medial longitudinal fasciculus, and bilateral intraaxial fasciculi of the facial nerve (Fig. 2). The histopathologic studies of Fisher (8) showing terminal bifurcation of a single paramedian artery to supply the dorsal pontine tegmentum bilaterally could explain why occlusion of a single vessel would produce these findings.FIG. 2: Schematic axial section through the caudal pons. The shaded area represents the site of the lesion that involves the right abducens nucleus, both medial longitudinal fasciculi, and both seventh (facial) nerve fasciculi.Jong Seok Bae, MD Hong Ki Song, MD Department of Neurology; Seoul Medical Center and College of Medicine, Hallym University; Seoul, Korea; E-mail: [email protected]

Unilateral Palpebral Ptosis Associated with Vinblastine Therapy

Vinblastine is a plant-derived alkaloid, that is clinically used as an antineoplastic drug. An important complication of vinca alkaloid therapy is neurotoxicity; the ocular system can be involved, with bilateral ophthalmoplegia, diplopia, and bilateral seventh nerve palsy with lagophthalmos. Palpebral ptosis is always bilateral and usually associated with vincristine therapy; rarely has it been reported in association with vinblastine. We report the case of a two-year-old child with systemic Langerhans' cell histiocytosis (LCH), who developed unilateral palpebral ptosis in his left eye six weeks after vinblastine therapy was initiated. This is the first case reported in the literature of unilateral palpebral ptosis during vinblastine therapy.

Oculomotor nerve paralysis and bilateral facial nerve paralysis as presenting signs of Lyme disease

Lyme disease is a tick-borne illness caused by the spirochete Borrelia burgdorferi . On occasions, patients with Lyme disease present with isolated cranial nerve palsies; multiple palsies have rarely been reported in the literature.We discuss the case of a patient, who presented with a third nerve palsy and bilateral seventh nerve palsies and was subsequently diagnosed as suffering from Lyme disease.To our knowledge, this is the first reported case of Lyme disease presenting with this combination of neurological signs. A 53-year-old male from the Scottish Western Isles presented with a two-day history of double vision. On examination, he was found to have a left third cranial nerve palsy and a left seventh cranial nerve palsy. Five days later, the patient developed a right seventh cranial nerve palsy. Serum and CSF enzymelinked immunosorbent assay (ELISA) and Western blot analysis were both positive for Lyme disease as was CSF polymerase chain reaction (PCR) analysis. Subsequently, the patient gave a history of multiple tick bites over the previous few years. He was commenced on appropriate antibiotic therapy and oral prednisolone. A rapid improvement in his condition was noted. This case demonstrates the importance of considering the diagnosis of Lyme disease in patients with multiple cranial neuropathies, especially those residing in, or with a history of travel to, endemic areas. In addition, it illustrates how early diagnosis, with the institution of appropriate therapy, leads to a prompt improvement in neurological symptoms and signs.