Neurosyphilis presenting as cranial nerve palsy, an entity which is easy to miss.

Neurosyphilis is a rare disease that until the 2000s was almost eradicated due to population awareness of HIV and efficient treatment. Since then, the prevalence of the entity is rising due to risk-associated behaviour such as unprotected intercourse. Neurosyphilis is still a difficult entity to diagnose especially when combined with acute HIV infection which can influence the usual clinical course of disease. In rare occasions, both acute HIV and early syphilis infection can present as mono or multiple cranial nerve palsies. This case demonstrates a rare manifestation of misdiagnosed early syphilis infection combined with acute HIV infection in a 34-year-old man with prior history of unprotected sex with men.

Cephalic tetanus: about two cases.

Tetanus is still a common problem in developing countries. Localized tetanus remains a rare clinical presentation dominated by cephalic localization. Head, neck or ear infection strongly guide the diagnosis. A trismus and one or several cranial nerve impairment are the major symptoms. We report two cases of cephalic tetanus associating a trismus with involvement of the cranial nerves. Diagnosis was difficult because the neurological damage preceded the trismus.

Orbital apex syndrome secondary to aspergilloma masquerading as a paranasal sinus tumor: A case report and literature review.

RATIONALE: Orbital apex syndrome is a complex clinical disorder featuring a collection of cranial nerve deficits characterized by impairment of the extraocular muscles, the ophthalmic branch of the trigeminal nerve, and even the optic nerve. Sino-orbital aspergillosis is rare but aggressive infection. Surgical resection accompanied by antifungal medication is advised currently. PATIENT CONCERNS: We report a 61-year-old woman diagnosed as aspergilloma presenting with the characteristic manifestations and imaging features of orbital apex syndrome. DIAGNOSES: Paranasal sinus tumor was misdiagnosed initially according to magnetic resonance imaging of the orbit. Finally aspergilloma was diagnosed by pathologic report. INTERVENTIONS: The anti-fungal medication, voriconazole, was administered immediately. Surgical excision was also done due to the poor response to medical treatment. OUTCOMES: Postoperative follow-up showed no recurrence of aspergillosis but the vision was lost permanently. LESSONS: Invasive sino-orbital aspergillosis as an aggressive disease with highly invasive patterns and it may be misdiagnosed as tumors. To achieve better prognosis and survival, clinicians should be aware of this distinct manifestation.

Cranial neuropathy and severe pain due to early disseminated Borrelia burgdorferi infection.

A 61-year-old man presented to the emergency department in the summer with a right seventh cranial nerve lower motor neuron palsy and worsening paraesthesias for 6 weeks. He had debilitating pain at the scalp and spine. Prior work up was unrevealing. The patient resided in the upper Midwest region of the USA and worked outdoors, optimising the landscape for white tailed deer. Repeat cerebrospinal fluid testing revealed a lymphocytic pleocytosis and positive IgM Lyme serology. Brain MRI demonstrated enhancement of multiple cranial nerves bilaterally. He was diagnosed with early Lyme neuroborreliosis and treated with 28 days of intravenous ceftriaxone. While the painful meningoradiculitis, also known as Bannwarth syndrome, is more commonly seen in Europe, facial palsy is more frequently encountered in the USA. Clinical manifestations of neuroborreliosis are important to recognise as the classic presentation varies by geography and on occasion repeat serological testing may be necessary.

Case Report: Neurobrucellosis with Plastered Spinal Arachnoiditis: A Magnetic Resonance Imaging-Based Report.

Diffuse spinal arachnoiditis in neurobrucellosis is a rare manifestation. We report a boy aged 17, presenting with hearing impairment and recurrent vomiting for 18 months, weight loss for 12 months, dysphagia, dysarthria, hypophonia for 6 months, and gait unsteadiness for 5 months. He had bilateral 5th (motor) to 12th cranial nerve palsy, wasting and weakness of limbs, fasciculations, absent tendon reflexes, and positive Babinski's sign. Cerebrospinal fluid (CSF) showed raised protein and pleocytosis. Magnetic resonance imaging (MRI) showed extensive enhancing exudates in cisterns and post-contrast enhancement of bilateral 5th, 6th, 7th, and 8th nerves. Spine showed clumping with contrast enhancement of the cauda equina roots and encasement of the cord with exudates. Serum and CSF were positive for anti-Brucella antibodies. He showed significant improvement with antibiotics. At 4 months follow-up, MRI demonstrated near complete resolution of cranial and spinal arachnoiditis. It is important to recognize such rare atypical presentations of neurobrucellosis.

Recurrent malignant otitis externa with multiple cranial nerve involvement: A case report.

Introduction: Necrotizing otitis externa is a rare but conditionally fatal infection of external auditory canal with extension to deep soft tissue and bones, resulting in necrosis and osteomyelitis of the temporal bone and scull base. This condition is also known as malignant otitis due to an aggressive behavior and poor treatment response. Early diagnosis of malignant otitis is a difficult challenge. We present an illustrative case of necrotizing otitis externa and suggest some strategies to avoid diagnostic and treatment pitfalls. Case Outline: A 70-year-old patient presented with signs of malignant otitis externa, complicated by peripheral facial palsy. Adequate diagnostic and treatment procedures were performed with clinical signs of resolution. The recurrence of malignant infection had presented three months after previous infection with multiple cranial nerve neuropathies and signs of jugular vein and lateral sinus thrombosis. An aggressive antibiotic treatment and surgery were carried out, followed by substantial recovery of the patient and complete restoration of cranial nerves' functions. Conclusion: Necrotizing otitis externa is a serious condition with uncertain prognosis. The suspicion of malignant external otitis should be raised in cases of resistance to topical treatment, especially in patient with predisposing factors. Evidence-based guideline for necrotizing otitis externa still doesn't exist and treatment protocol should be adjusted to individual presentation of each patient.

[Acute cranial multineuritis caused by a fungus]. / Multineuritis craneal aguda de causa fúngica.

TITLE: Multineuritis craneal aguda de causa fungica.

Gradenigo's syndrome: is fusobacterium different? Two cases and review of the literature.

Gradenigo's syndrome is a rare but life threatening complication of acute otitis media (AOM), which includes a classic triad of otitis media, deep facial pain and ipsilateral abducens nerve paralysis. The incidence of Fusobacterium necrophorum infections has increased in recent years. We describe two cases of Gradenigo's syndrome caused by F. necrophorum. Additional four cases were identified in a review of the literature. Gradenigo's syndrome as well as other neurologic complications should be considered in cases of complicated acute otitis media. F. necrophorum should be empirically treated while awaiting culture results.

Clinical and imaging findings in patients with neurosyphilis: a study of a cohort and review of the literature.

BACKGROUND: Most patients with neurosyphilis are considered asymptomatic. The diagnosis is challenging and the role of neuroimaging is not yet well established. The present study was conducted to focus on the clinical findings and further characterize the imaging features of the disease, along with a review of the pertinent literature. METHODS: Six male patients with neurosyphilis based on abnormal cerebrospinal fluid findings, five of whom were asymptomatic at presentation, underwent cranial computerized tomography (CT) and magnetic resonance imaging (MRI). They also underwent a complete physical, neurological, and ophthalmological examination, with special attention paid to atherosclerotic vascular risk factors. In addition, all were examined for cardiac involvement using electrocardiography and cardiac ultrasound. RESULTS: The meticulous neurological and ophthalmological examination revealed abnormalities in five patients, most commonly cranial nerve involvement (three patients) and hemiparesis (two patients). The CT and MRI studies revealed abnormalities in five of the six patients, and in all six patients, respectively. The most common findings were brain infarcts, which were demonstrated in four of the six patients. MRI was found to be more sensitive than CT in detecting these brain infarcts, as expected. CONCLUSIONS: Vascular insult was the most common neuroimaging finding in our patients with neurosyphilis, probably due to meningovascular endarteritis. Neurosyphilis should always be considered in young patients with unexplained brain infarcts.

Perineural infiltrates in Pseudomonas keratitis.

We describe 2 cases of contact lens-related microbial keratitis caused by infection with Pseudomonas aeruginosa in which perineural infiltrates were observed at presentation. In both cases, examination by confocal microscopy was negative for Acanthamoeba cysts but bacterial cultures and microscopy of corneal scrapings were positive for P aeruginosa. Both cases responded rapidly to treatment with topical levofloxacin with no significant long-term sequelae. These observations indicate that perineural infiltrates may occur in Pseudomonas keratitis without underlying Acanthamoeba infection and are, therefore, not pathognomonic of Acanthamoeba infection.

[Invasive aspergillosis of sphenoidal sinus in a patient in Djibouti, revealed by palsy of cranial nerves: a case report]. / Aspergillose invasive du sinus sphénoïdal révélée par une paralysie de paires crâniennes : à propos d'un cas pris en charge à Djibouti.

The authors report a case of invasive aspergillosis of a sphenoid sinus mucocele revealed in a patient with diabetes in Djibouti by homolateral palsy of the 3rd, 4th, 5th and 6th nerves. This rare condition occurs preferentially in immunodeficient subjects. Because of its clinical polymorphism, its diagnosis is difficult and is often not made until complications develop. Endonasal surgery with anatomopathological and mycological examination is both a diagnostic and therapeutic procedure. It must be performed early, to avoid functional or even life-threatening complications.

Cranial nerve palsy as a factor to differentiate tuberculous meningitis from acute bacterial meningitis.

Tuberculous meningitis (TBM) and acute bacterial meningitis (ABM) cause substantial mortality and morbidity in both children and adults. Identification of poor prognostic factors at patient's admission could prepare physicians for more aggressive monitoring of patients with meningitis. The objective of this study was to determine the predictive value of neurological features to differentiate ABM and TBM. A retrospective study was conducted between patients affected with ABM or TBM admitted to three teaching hospitals during the last 14 years in Zahedan the central city of Sistan and Balouchestan province (Iran). The neurological features include seizure, level of consciousness, stroke, focal neurologic deficit and cranial nerve palsy at the time of admission. Mean age for patients with TBM and ABM were 41 ± 22.4 and 24 ± 18.5 years respectively. In univariate analysis, all measured variables revealed significant difference between ABM and TBM patients except for seizure episodes. Multivariate logistic regression analysis showed positive predictive effect of cranial nerve palsy (AOR=1.980, CI 95%: 1.161-3.376) on the diagnosis of TBM. In our study cranial nerve palsies was the most important neurological predictor factor to differentiate TBM from ABM.

Rhino-orbito-cerebral mucormycosis: five cases.

INTRODUCTION AND OBJECTIVE: Mucormycosis is an invasive fungal infection usually observed in immunocompromised patients. Mucormycosis is rapidly fatal without an early diagnosis and treatment. We report five patients of rhino-orbital-cerebral mucormycosis and a literature review. DESIGN: The medical records of five patients presenting with rhino-orbital-cerebral mucormycosis, admitted between January 1995 and December 2007, were analyzed. All patients underwent tissue biopsy. The histologic sections revealed the presence of non-septate hyphae of the order Mucorales. RESULTS: The five patients, three men and two women, between 27 and 61 years of age, were all diabetic. The main symptoms were exophthalmia (five patients), facial swelling (four patients), periorbital cellulitis (four patients), and cranial nerve palsy (four patients). Anterior rhinoscopy revealed palatine or nasal necrotic lesions in four patients. All presented with diabetic ketoacidosis and CT scan revealed rhino-orbital-cerebral involvement in every patient. All patients were given intravenous amphotericin B. Four patients underwent surgical debridement of necrotic tissue. Two patients survived. CONCLUSIONS: Mucormycosis is usually a fatal infection in diabetic patients. Early diagnosis should be based on imaging data and histology. Amphotericin B must be rapidly initiated and associated with aggressive surgical debridement to reduce mortality.

Incidence, predictors and prognostic value of cranial nerve involvement in patients with tuberculous meningitis: a retrospective evaluation.

BACKGROUND: Cranial nerve involvement is commonly observed in patients with tuberculous meningitis. The present study evaluated the incidence, predictors and prognostic significance of cranial nerve involvement in tuberculous meningitis. MATERIALS AND METHOD: One hundred-fifty-eight adult patients with tuberculous meningitis were retrospectively evaluated and followed up for 9 months. A detailed clinical evaluation and cranial magnetic resonance imaging were done in every patient. RESULT: At inclusion, 60 (38%) patients had cranial neuropathy. Sixteen patients were having involvement of two or more cranial nerves. Abducent nerve was the most frequently (32.3%) affected cranial nerve. Predictors of cranial nerve involvement were age >25 years, history of vomiting, altered sensorium, hemiparesis, diplopia, papilledema, signs of meningeal irritation, severe functional disability, cerebrospinal fluid protein >2.5 g/L and cerebrospinal fluid cell count >100/mm(3). The presence of optochiasmatic arachnoiditis and hydrocephalus was also a significant predictor of cranial neuropathy. Presence of cranial neuropathy was significantly associated with poor outcome. CONCLUSION: Cranial nerve involvement occurred in more than one third of patients with tuberculous meningitis. The presence of cranial neuropathy was associated with poor outcome.

[Central nervous system tuberculosis in adult patients]. / Felnottek központi idegrendszeri tuberkulózisa.

UNLABELLED: Central nervous system tuberculosis is the fifth most frequent and at the same time most severe form of extrapulmonary tuberculosis diseases. It presents with no typical signs, thus early diagnosis and treatment is of high importance concerning the outcome. Authors present the characteristics, diagnostic and therapeutic alternatives of central nervous system tuberculosis through a case report and a retrospective study of 15 patients. PATIENTS AND METHODS: Authors performed a retrospective analysis of medical records of patients with central nervous system tuberculosis in an academic teaching hospital (Department of Neurology and Infectious Diseases, United Szent István-Szent László Hospital, Budapest, Hungary). RESULTS: Median age of patients was 54.5 years, and 6 (40%) were females. Cerebrospinal fluid findings at admission showed elevated protein (1.54 g/l; 95% confidence interval (CI): 1.01-2.05), cell count (mean: 337/µl; CI: 171.9-502.5), and decreased glucose index (0.32; CI: 0.15-0.52). 14 patients (93.3%) had hyponatremia. Average duration of symptoms were 16.3 days (1-40). On physical examination meningeal irritation was absent in 9 patients (60%). On admission headache and altered consciousness was present in 53%, while headache, fever, nuchal rigidity was present in only 33.3%. Diagnosis was culture and/or PCR confirmed in 46.7% of the cases. Two third of patients were followed-up at least for one year, and nine patients presented neurological sequel. Authors found that patients with central nervous system tuberculosis present with unspecific symptoms, but later progressive disorientation, cranial nerve palsies and convulsions may develop. Headache and altered consciousness proved to be the leading symptoms among these patients. Even today, diagnostic gold standard procedure is cultivating M. tuberculosis on solid and liquid medium. The polymerase chain reaction, which is known to have sensitivity between 27% and 86%, was positive in two of eight samples. Revealing predisposing factors (immunodeficiency, HIV infection, previous tuberculosis exposure) promotes setting up early diagnosis. Co-administration of four antituberculotic drugs for 12 months cured all patients, but authors note that even in cases with early diagnosis and optimal treatment various neurological impairment and seldom death can occur. CONCLUSIONS: Central nervous system tuberculosis is a rare but regularly emerging disease with unspecific signs and symptoms. The diagnosis may be difficult. It should be considered as a differential diagnostic issue in patients with uncharacteristic subacute conditions with headache, disorientation, elevated protein and low glucose in cerebrospinal fluid.

Role of imaging in the diagnosis of acute bacterial meningitis and its complications.

Acute bacterial meningitis is a common neurological emergency and a leading cause of death and neurological disability worldwide. Diagnosis is based on clinical and microbiological findings with neuroimaging in the form of CT reserved for those with specific adverse clinical features or when an underlying cause such as mastoiditis is suspected. MRI is extremely useful for detecting and monitoring the complications of meningitis. These can be remembered by the mnemonic HACTIVE (hydrocephalus, abscess, cerebritis/cranial nerve lesion, thrombosis, infarct, ventriculitis/vasculopathy and extra-axial collection). Diffusion weighted imaging (DWI) and magnetic resonance spectroscopy (MRS) are useful to distinguish abscess from other ring enhancing lesions.

Neurological complications in two children with Lemierre syndrome.

Lemierre syndrome is a distinct clinical syndrome comprising oropharyngeal sepsis and fever, internal jugular vein thrombosis and remote septic metastases caused by Fusobacterium species. The mortality rate was historically high and although use of antibiotics led to a dramatic fall in incidence, a resurgence has been seen recently. A 14-year-old male developed Lemierre syndrome after tonsillitis. There was extensive leptomeningitis, especially over the clivus, causing 6th and 12th cranial nerve palsies, a clinical feature termed the 'clival syndrome'. He also developed an epidural abscess in the cervical spine, which was unsafe for surgical drainage. Conservative treatment with an extended course of antibiotics and anticoagulation for jugular vein thrombosis led to a good recovery. A 15-year-old female developed Lemierre syndrome after a persistent sore throat lasting 7 weeks. She had palsy of the 12th cranial nerve from clival osteomyelitis. She was treated with a 6-week course of antibiotics and anticoagulants leading to almost full recovery at 3-month review. Awareness of the potential neurological complications of Lemierre syndrome and prompt management are crucial in reducing morbidity and mortality in this 'forgotten disease'.