Anti-hu paraneoplastic syndrome presenting as bilateral sixth cranial nerve palsies.

A 62-year-old woman presented with diplopia caused by bilateral sixth cranial nerve palsies. Two weeks later, she had bulbar weakness and ataxia. Brain magnetic resonance imaging showed non-specific abnormalities and spinal fluid was acellular but contained an elevated protein and oligoclonal bands. A paraneoplastic screen showed anti-Hu antibodies. Her clinical condition improved with immunoglobulin and systemic corticosteroid treatment. Breast cancer was diagnosed 21 months later by mammography but there were no metastases detected. Four and half years after the onset of her diplopia, she died of diffuse metastatic breast cancer. This is the first reported case of anti-Hu paraneoplastic brain stem encephalitis presenting with sixth cranial nerve palsies.     

Chronic isolated sixth nerve palsies

Twenty-seven cases of chronic isolated abduction deficit, seen in 25 years of neuro-ophthalmology consultation, were reviewed. "Isolated" was defined as the lack of any other neurologic or ophthalmologic findings for at least six months. Cases were further subdivided into the categories of true and pseudo-abducens paresis. The origin, diagnosis, and management of long-standing abducens palsies were determined. Cases of pontine glioma, chordoma, chondrosarcoma, and meningioma that presented as an isolated abducens palsy and remained undiagnosed for at least six months were reviewed.     

A case of bilateral simultaneous sixth cranial nerve palsies secondary to diabetes mellitus.

Ocular motor cranial nerve palsy secondary to diabetes mellitus usually affects one cranial nerve at a time. We report a patient with simultaneous bilateral sixth nerve palsies attributed to diabetes. Although an extremely rare cause of this phenomenon, diabetes may be the explanation after other causes have been excluded.     

Third nerve palsies

The diagnosis and management of third nerve dysfunction varies according to the age of the patient, characteristics of the third nerve palsy, and presence of associated symptoms and signs. Indeed, third nerve palsies may be partial or complete, congenital or acquired, isolated or accompanied by signs of more extensive neurological involvement. They can result from lesions located anywhere from the oculomotor nucleus to the termination of the third nerve in the extra-ocular muscles within the orbit. Recent advances in noninvasive neuroimaging facilitate early diagnosis; however, management of a patient presenting with an isolated third nerve palsy remains a challenge.     

Third nerve palsies

The diagnosis and management of third nerve dysfunction vary according to the age of the patient, the characteristics of the third nerve palsy, and the presence of associated symptoms and signs. Third nerve palsies can result from lesions located anywhere from the oculomotor nucleus to the termination of the third nerve in the extraocular muscles within the orbit, and may be the herald manifestation of underlying neurological emergencies such as intracranial aneurysm, pituitary apoplexy, and giant cell arteritis. Recent advances in noninvasive neuroimaging facilitate early diagnosis, but the management of a patient presenting with isolated third nerve palsy remains a challenge.     

Bilateral sixth cranial nerve palsy following lumboperitoneal shunt

Lumboperitoneal (LP) shunt is considered as a unique procedure in neurosurgical practice. The over drainage of Cerebrospinal fluid (CSF) is considered a usual side effect of the procedure that usually manifests in the form of subdural collection, headache, or nausea and vomiting. However, cranial nerve palsy, particularly abducens nerve palsy, is a rare manifestation of CSF over drainage. We describe our experience with a patient that has developed bilateral sixth nerve palsy followed by LP shunt insertion, with resolution of the symptoms upon removal of the shunt. This shades the light on a rare but significant complication related to Lumboperitoneal (LP) shunt insertion that necessitate the modification in the hardware of valve or insertion technique to decrease the risk of CSF over the drainage.

Lumboperitoneal (LP) shunt, in neurosurgical practice, is a unique procedure. ¹ Cerebrospinal fluid (CSF) over drainage is an often-encountered side effect of this procedure along with shunt failure. ¹ Cerebrospinal fluid over drainage can manifest as headache, subdural collection, subdural hematoma, or epidural hematoma. ² Abducens palsy is a rare complication of various CSF diversion procedures. ³ Sporadic case reports, case series, and systematic reviews have discussed cases of abducens palsy following diagnostic LP shunt, lumbar drain, spinal/epidural block and ventriculoperitoneal (VP) shunt. ² However, there has been no evidence of any such case in the past literature about bilateral sixth cranial nerve palsy following by LP shunt. We describe our experience with the recognition, management, and follow-up of a patient having bilateral sixth nerve palsy following by LP shunt insertion. ^2,4 These shades the light on a rare but significant complication related to Lumboperitoneal (LP) shunt insertion.     

Familial recurrent cranial nerve palsies

Family cases of recurrent cranial nerve palsies are seldom reported. This paper presents a family with recurrent facial and ocular nerve palsies in 2 brothers. Their father and his sister had Bell's palsies. Examinations provided no explanation. Six previous reports of families with recurrent cranial nerve palsies are summarized. The pedigrees speak in favour of an autosomal dominant mode of inheritance of predisposing factors. The pathogenetic mechanism might be vascular or autoimmune, but is still unknown.     

Cognitive impairment following closed head injury.

Cognitive impairments are usually the most disabling sequelae of CHI. The earliest stage of recovery from moderate to severe closed head injury is a period of PTA that typically includes memory loss for events preceding and surrounding the injury and memory loss for events occurring since the injury. Following resolution of PTA, deficits may be present in a number of cognitive domains. Memory and attention/information processing speed and efficiency are typically the cognitive domains most severely affected by head injury. Intellectual, language, and perceptual skills tend to be relatively preserved. Neurologic variables such as pupillary reactivity and worst GCS score are prognostic of cognitive impairment at 1 to 2 years postinjury. Following mild head injury, impairments of memory and information processing may be apparent within the first week of recovery. These deficits usually resolve in 1 to 3 months, although chronic complaints persist in a minority of individuals. The long-term cognitive effects of CHI are typically more severe for younger children than older children. Neuropsychologic assessment provides an objective way to measure the presence and severity of cognitive impairment.     

Identical twins with papilloedema and cranial nerve palsies.

Central nervous system involvement has not been reported previously as a manifestation of sarcoidosis in monozygotic twins. Twin sisters developed multiple cranial nerve palsies and papilloedema within a two month period. Clinical features and the course of their diseases were remarkably similar.     

Isolated cranial nerve palsies due to brainstem lesions.

Isolated cranial nerve palsies are often attributed to lesions of the respective nerves along their extraaxial courses. There are a significant number of reports of individual patients with cranial nerve palsies, mostly of the 3rd and 6th nerves, as the sole manifestation of brainstem lesions proven by magnetic resonance imaging (MRI) or computer-assisted tomography (CT). An intraaxial basis may still be underestimated if based on MRI only, as electrophysiological abnormalities indicating brainstem lesions (masseter reflex, blink reflex, DC electrooculography) may be independent from MRI-documented morphological lesions. This article reviews the evidence that ischemic and demyelinating brainstem lesions are an important and underestimated cause of clinically isolated cranial nerve palsies. Especially in middle-aged and elderly people with 3rd and 6th nerve palsies, small pontine and mesencephalic infarctions seem to be more frequent than small-vessel ischemic infarctions of the extraaxial nerves.     

Pontine infarction manifesting as isolated cranial nerve palsies.

Isolated ipsilateral fifth, seventh, tenth and twelfth cranial nerve palsies in a 74-year-old woman were shown at autopsy to result from an inferior lateral pontine infarction. The clinical features and pathogenesis of this uncommon lesion are discussed.