Isolated Cranial Nerve VI Palsy and Neurosyphilis: A Case Report and Review of Related Literature.

An isolated cranial nerve VI palsy is a rare initial manifestation of undiagnosed neurosyphilis. A 33-year-old male presented with a one month history of progressive headache and diplopia. Neurologic examination only revealed an isolated abducens palsy on the left. Cranial imaging was unremarkable. Examination of his cerebrospinal fluid revealed lymphocytic predominant leukocytosis and elevated protein. Microbiologic work-up were all negative. Further work-up revealed the patient to be serum Rapid Plasma Reagin and Enzyme Immunoassay reactive. Enzyme-linked immunosorbent assay for Human Immunodeficiency Virus also tested positive. His cerebrospinal fluid was then sent for Rapid Plasma Reagin to confirm the diagnosis of neurosyphilis. He completed 14 days of intravenous penicillin and was eventually discharged with partial resolution of the abducens palsy. We describe the second case of neurosyphilis presenting only with an isolated cranial nerve VI involvement. On further review, ours was the first case documented on an individual who had an undiagnosed Human Immunodeficiency Virus infection. There are various differentials for an isolated cranial neuritis but infectious causes, particularly neurosyphilis, should be considered among young individuals with known risk factors despite their apparently benign medical history.

Wernicke-Korsakoff Syndrome in Hyperemesis Gravidarum: A Case Report and Literature Review.

BACKGROUND: Wernicke-Korsakoff syndrome (WKS) is a triad of ophthalmoplegia, ataxia and memory deficits due to thiamine insufficiency resulting from under- or untreated Wernicke's encephalopathy (WE), which may be associated with hyperemesis gravidarum (HEG). CASE PRESENTATION: We present a case of a 36-year-old Filipino woman in her first trimester with HEG, along with the WKS triad and abnormal hyperintense signals in the bilateral thalami and midbrain, left occipital lobe, periaqueductal gray matter and pontine periventricular areas. Neurologic deficits partially improved but persisted despite intravenous thiamine administration. EVIDENCE REVIEW: A review of current treatments for WE, and the prevention and neurocognitive recovery of WKS was done. The beneficial effects of thiamine for acute WKS are supported by several case reports and clinical experience. Evidence from one randomized controlled trial wherein thiamine was given in various doses for treating WE or preventing WKS in an alcohol-dependent population is limited by methodological issues. Rehabilitation and pharmacotherapy for neurocognitive recovery seems promising, but they have inadequate evidentiary support. More robust studies on multi-modal strategies are warranted to facilitate the neurocognitive recovery of patients with WKS.

Rare case of intramedullary spinal cord metastasis from nasopharyngeal carcinoma.

INTRODUCTION: Central nervous system (CNS) metastasis from nasopharyngeal carcinoma (NPC) is rare and presents with high morbidity and mortality. As a rare entity, a few patients with CNS metastasis from NPC have been reported, and no studies were available on treatment and prognosis. Based on our clinical experience, early diagnosis with incorporation of a clear palliative plan is imperative in providing holistic care for patients with locally-invasive and metastatic nasopharyngeal carcinoma. CASE REPORT: Our study reports a case of a 48-year-old Filipino male with known NPC Stage IVB who developed acute symptoms of constipation, urinary retention, and bilateral lower limb weakness and numbness. Magnetic resonance imaging showed intramedullary lesions in multiple segments of the spinal cord. Steroid and radiotherapy of the spine were initiated with noted transient improvement of the motor strength. Subsequently, he developed cancer-related stroke. The patient progressively deteriorated despite best medical care.

Thrombolysis Outcomes in Patients with Diabetes and Previous Stroke: A Meta-Analysis.

BACKGROUND: Intravenous tissue-type plasminogen activator (IVtPA) is a proven treatment for acute ischemic stroke; however, diabetes mellitus (DM) and previous cerebral infarction (PCI) were considered relative contraindications for thrombolysis within the 3-4.5 h period. OBJECTIVE: The study aimed to determine the safety and efficacy of IVtPA among diabetic patients with PCI presenting with acute ischemic stroke. METHODS: Studies which evaluated the outcome of IVtPA in terms of symptomatic intracerebral hemorrhage (sICH), functional outcome in modified Rankin scale, and death among diabetic patients with PCI presenting with acute ischemic stroke within the 3-4.5 h period were systematically searched until July 2019. Screening and eligibility criteria were applied. Risk of bias was evaluated using the Newcastle-Ottawa Scale. Odds ratios (ORs) with 95% confidence interval (CI) were used to compare measures of treatment effect. Mantel-Haenszel method and random-effects model were also employed. RESULTS: Four registry-based studies with a total of 44,572 patients were included for quantitative synthesis. Giving IVtPA among DM+/PCI+ patients did not result in significantly increased rate of sICH (OR, 1.09; 95% CI, 0.88, 1.36) compared to No DM+/PCI+ patients. However, there was significantly higher mortality (OR, 1.81; 95% CI, 1.60, 2.06) in the DM+/PCI+ group. Conversely, among those who survived, the DM+/PCI+ patients were more functionally independent at 3 months (OR, 0.76; 95% CI, 0.61, 0.94). CONCLUSION: Limited evidence suggests that thrombolysis in DM+/PCI+ patients does not result in significantly higher incidence of sICH and may improve functional independence. However, the significantly higher mortality in this group warrants an assessment of the individualized risk-benefit ratio in the use of IVtPA.

Secondary hypokalemic paralysis with bulbar weakness and reversible electrophysiologic abnormalities: A case report and systematic review.

Hypokalemic periodic paralysis secondary to distal renal tubular acidosis presenting with prominent bulbar symptoms is extremely rare. The exact pathophysiology by which hypokalemia causes weakness is yet to be elucidated though muscle and nerve membrane hyperpolarization have been hypothesized. The pathophysiology of bulbar involvement in this condition is even more unclear. We report a case presenting as acute flaccid quadriplegia with prominent bulbar symptoms that reversed once potassium levels returned to normal. Serial nerve conduction studies were performed at various potassium levels revealing electrophysiologic abnormalities that corrected with potassium repletion. A systematic review of the literature was also conducted focusing on bulbar symptoms and electrophysiologic findings in hypokalemic periodic paralysis. Nerve conduction abnormalities in this condition are seldom documented, but reports have shown reduced amplitudes of compound motor action potentials and abnormal F-waves during acute attacks of hypokalemic paralysis.

Shunt Surgery for Neurocutaneous Melanosis with Hydrocephalus: Case Report and Review of the Literature.

BACKGROUND: Neurocutaneous melanosis is a rare phakomatosis characterized by large or multiple pigmented nevi and melanosis of the leptomeninges. It is often complicated by hydrocephalus due to melanotic deposits interfering with cerebrospinal fluid reabsorption in the basal cisterns or causing foraminal or aqueductal obstruction. In 10% of cases, it will be associated with the Dandy-Walker complex. CASE DESCRIPTION: We present the case of a 6-year-old girl with multiple congenital hairy nevi presenting with generalized tonic-clonic seizures, headache, and vomiting. Neuroimaging showed a communicating hydrocephalus associated with the Dandy-Walker variant, melanotic deposits in the amygdalae, thalami, and cortical sulci, and abnormal leptomeningeal enhancement. After undergoing ventriculoperitoneal shunt insertion, the symptoms of increased intracranial pressure abated. However, she again showed deterioration 1 month postoperatively due to progressive leptomeningeal spread suspicious for malignant degeneration. CONCLUSION: The results of the present case and the findings from a review of related data suggest that shunt insertion (ventriculoperitoneal or cystoperitoneal) is an effective palliative measure for patients with neurocutaneous melanosis with associated hydrocephalus. Despite treatment, however, the prognosis of these patients remains poor owing to malignant progression and leptomeningeal spread of lesions, in particular, in cases associated with the Dandy-Walker complex.

Clinical Profile and Outcome of Postthymectomy versus Non-Thymectomy Myasthenia Gravis Patients in the Philippine General Hospital: A 6-Year Retrospective Study.

BACKGROUND: Myasthenia gravis is an autoimmune neuromuscular disorder characterized by the production of abnormal autoantibodies directed against the receptors present in the neuromuscular junction. It has been the standard practice to offer thymectomy in all generalized myasthenia gravis patients despite the lack of robust evidence. OBJECTIVES: The objectives of this study are to describe the clinical profile and differentiate the clinical outcomes of thymectomy versus non-thymectomy and thymomatous versus non-thymomatous myasthenia gravis patients in the Philippine General Hospital. METHODOLOGY: Between 2009 and 2014, a total of 69 postthymectomy and 16 non-thymectomy patient records were successfully retrieved. The demographic characteristics, surgical approach, and histopathologic results were obtained. The clinical outcome after 6 months or 1 year-follow-up was also determined and grouped according to the following: (1) complete remission, (2) pharmacological remission, (3) no clinical change, (4) worsening symptoms, and (5) mortality. RESULTS: Majority of the patients were females (68.0%) with a mean age of 39.8 years and a mean duration of myasthenic symptoms of 21 months. Using the Myasthenia Gravis Foundation of America classification, 54.1% of patients fell under Class II and 48.2% of them presented with generalized weakness. In this study, 60.8% of postthymectomy myasthenia gravis patients had either complete remission or pharmacologic remission compared with 12.5% among non-thymectomy patients (p-value <0.001). No significant difference in the clinical outcome was found between thymomatous and non-thymomatous myasthenia gravis after thymectomy (p-value = 0.29). CONCLUSION: This study showed that both thymomatous and non-thymomatous myasthenia gravis patients who underwent thymectomy had a higher incidence of complete stable remission and pharmacologic remission as compared with myasthenia gravis patients who did not undergo thymectomy.