Idiopathic Bilateral Simultaneous Facial Nerve Palsy (B-FNP)

Background: Bilateral facial nerve palsy (B-FNP) is a rare clinical manifestation with incidence of 1 per 5 million people. Furthermore, it approximately accounts for 0.3 – 2 % of facial palsy cases. This B-FNP case is intricate in the diagnosis, finding the aetiology, and treatment that need hospitalization. Case Report: A male 64-year-old with bilateral facial nerve palsy that happened suddenly followed by difficulty in closing both eyes and facial abnormality without clear cause within 7 days of onset. Risk factor is hypertension stage 2. During neurological examination, there was bilateral peripheral facial nerve palsy grade IV in right side and grade III in the left side (House Brackmann grading system) that was not followed by other cranial nerve abnormality and motoric examination is normal. Supporting examinations such as lumbar puncture, thorax photo, and head MRI with contrast shows normal result. ENMG examination shows absent of blink reflex. Prednisone 60 mg orally was given with tapering off dosage 10 mg per day. Patient was hospitalized for 12 days and was discharge with good clinical improvement with bilateral peripheral facial nerve paralysis grade II in right side and grade I in left side. Conclusion: Bilateral facial nerve paralysis is a rare clinical manifestation and challenging in diagnosis. It is important to have a differential diagnosis in cases with bilateral cranial nerve palsy. Careful physical examination and appropriate supporting examinations such as laboratory and radiology in necessary to evaluate the underlying cause

Two Immigrants with Tuberculosis of the Ear, Nose, and Throat Region with Skull Base and Cranial Nerve Involvement

We report two immigrants with tuberculosis of the skull base and a review of the literature. A Somalian man presented with bilateral otitis media, hearing loss, and facial and abducens palsy. Imaging showed involvement of both mastoid and petrous bones, extending via the skull base to the nasopharynx, suggesting tuberculosis which was confirmed by characteristic histology and positive auramine staining, while Ziehl-Neelsen staining and PCR were negative. A Sudanese man presented with torticollis and deviation of the uvula due to paresis of N. IX and XI. Imaging showed a retropharyngeal abscess and lysis of the clivus. Histology, acid-fast staining, and PCR were negative. Both patients had a positive Quantiferon TB Gold in-tube result and improved rapidly after empiric treatment for tuberculosis. Cultures eventually yielded M. tuberculosis. These unusual cases exemplify the many faces of tuberculosis and the importance to include tuberculosis in the differential diagnosis of unexplained problems

Serum and cerebrospinal fluid brain damage markers neurofilament light and glial fibrillary acidic protein correlate with tick-borne encephalitis disease severity—a multicentre study on Lithuanian and Swedish patients

Background and purpose: Our aim was to examine the correlation between biomarkers of neuronal and glial cell damage and severity of disease in patients with tick-borne encephalitis.// Methods: One hundred and fifteen patients with tick-borne encephalitis diagnosed in Lithuania and Sweden were prospectively included, and cerebrospinal fluid (CSF) and serum samples were obtained shortly after hospitalization. Using pre-defined criteria, cases were classified as mild, moderate or severe tick-borne encephalitis. Additionally, the presence of spinal nerve paralysis (myelitis) and/or cranial nerve affection were noted. Concentrations of the brain cell biomarkers glial fibrillary acidic protein (GFAP), YKL-40, S100B, neurogranin, neurofilament light (NfL) and tau were analysed in CSF and, in addition, NfL, GFAP and S100B levels were measured in serum. The Jonckheere-Terpstra test was used for group comparisons of continuous variables and Spearman's partial correlation test was used to adjust for age.// Results: Cerebrospinal fluid and serum concentrations of GFAP and NfL correlated with disease severity, independent of age, and with the presence of nerve paralysis. The markers neurogranin, YKL-40, tau and S100B in CSF and S100B in serum were detected, but their concentrations did not correlate with disease severity.// Conclusions: Neuronal cell damage and astroglial cell activation with increased NfL and GFAP in CSF and serum were associated with a more severe disease, independent of age. Increased GFAP and NfL concentrations in CSF and NfL in serum were also indicative of spinal and/or cranial nerve damage. NfL and GFAP are promising prognostic biomarkers in tick-borne encephalitis, and future studies should focus on determining the association between these biomarkers and long-term sequelae

Emlőtumor intracranialis metasztázisainak eredményes szisztémás palliatív kemoterápiája | Effective systemic palliative chemotherapy for intracranial metastases of breast cancer

Absztrakt A szerzők két esetet mutatnak be. A 69 éves nőbetegnél helyileg előrehaladott és pulmonalis, illetve multiplex cerebralis metasztázisokat adó triple negatív ductalis emlődaganatot diagnosztizáltak. A tünetmentes cerebralis metasztázisok komplett radiológiai remisszióját észlelték szisztémás carboplatin-docetaxel (75 mg/m2) kemoterápia mellett. A beteg később palliatív koponyabesugárzást és 2. vonalú kemoterápiát kapott. A diagnózistól számítva másfél évet élt, részlegesen megőrzött önállósággal. Az 57 éves nőbetegnél meningitis carcinomatosát, nyirokcsomó- és multiplex ossealis metasztázist adó hormonérzékeny lobularis emlődaganatot mutattak ki. A nyirokcsomó-metasztázis megjelenése előtt intrathecalis methotrexat kemoterápiát alkalmaztak, amely mellett neurológiai tünetei megszűntek. A zsigeri metasztázis kifejlődésekor szisztémás ifosfamid (1000 mg/m2, 1–3. nap) – etoposid (100 mg/m2, 1–3. nap) kemoterápiát kapott, amely mellett a nyirokcsomóáttét komplett klinikai remisszióját és a neurológiai tünetmentesség stabilitását észlelték. A meningitis carcinomatosa diagnózisától számítva több mint egy évet élt, önálló életvitellel. Mindkét eset a szisztémás kemoterápia lehetséges hatásosságát mutatja intracranialis metasztázisokat adó emlődaganatok kezelésében. Orv. Hetil., 2016, 157(45), 1809–1813. | Abstract The authors present the history of two patients. The first patient, a 69-year-old woman was diagnosed with locally invasive triple negative breast cancer with pulmonary and cerebral metastases. Complete radiological remission of the clinically asymptomatic cerebral metastases was detected under systemic chemotherapy with carboplatin-docetaxel (75 mg/m2). Later, the patient received whole brain radiotherapy and a second line of chemotherapy. The overall survival was 20 months from the diagnosis of cerebral metastases with conservation of partial autonomy. The second patient, a 57-year-old woman was diagnosed as having hormone sensitive lobular breast cancer with leptomeningeal, lymphonodular and multiple osseal metastases. Before the appearance of the lymphonodular metastasis the patient received intrathecal methotrexate chemotherapy for the leptomeningeal carcinomatosis. Her neurological symptoms completely disappeared. At the onset of the lymphonodular metastasis systemic chemotherapy with ifosfamide (1000 mg/m2, D1–3) – etoposide (100 mg/m2, D1–3) was started allowing complete clinical remission of the lymphadenomegaly and stability of the asymptomatic neurological status. The overall survival was 13 months from the diagnosis of leptomeningeal carcinomatosis with conservation of autonomy. The two cases support potential efficacy of systemic chemotherapy for intracranial metastases of breast cancer. Orv. Hetil., 2016, 157(45), 1809–1813

Contrasting disease patterns in seropositive and seronegative neuromyelitis optica: A multicentre study of 175 patients

BACKGROUND: The diagnostic and pathophysiological relevance of antibodies to aquaporin-4 (AQP4-Ab) in patients with neuromyelitis optica spectrum disorders (NMOSD) has been intensively studied. However, little is known so far about the clinical impact of AQP4-Ab seropositivity. OBJECTIVE: To analyse systematically the clinical and paraclinical features associated with NMO spectrum disorders in Caucasians in a stratified fashion according to the patients' AQP4-Ab serostatus. METHODS: Retrospective study of 175 Caucasian patients (AQP4-Ab positive in 78.3%). RESULTS: Seropositive patients were found to be predominantly female (p 1 myelitis attacks in the first year were identified as possible predictors of a worse outcome. CONCLUSION: This study provides an overview of the clinical and paraclinical features of NMOSD in Caucasians and demonstrates a number of distinct disease characteristics in seropositive and seronegative patients

Bell's Palsy - Medical Treatment and Influence of Prognostic Factors

Bell’s palsy is an acute onset of peripheral facial nerve dysfunction. Despite extensive research the aetiology is unclear. The most prevailing theory is that reactivation of viruses such as Herpes simplex type-1 or Varicella zoster may cause Bell’s palsy. The natural course is favourable but at least 30% of patients will suffer sequelae such as residual paresis, synkinesis and/or contracture. The most commonly used medical treatment to reduce sequelae has been corticosteroids and/or antiviral agents, the usefulness of which has been debated since earlier studies have shown diverging results. The aim of this thesis was to evaluate the effect of treatment with prednisolone and/or valaciclovir in a large number of Bell’s palsy patients and study how prognostic factors such as age, severity of palsy at baseline, and time to treatment start may influence outcome. Data from the Scandinavian Bell’s Palsy Study (SBPS), a prospective, randomised, double-blind, placebo-controlled multicentre study with 12-month follow-up, were evaluated. In total, 829 patients, aged 18−75, were included in the intention to treat (ITT) analysis: 210 patients received prednisolone plus placebo, 207 valaciclovir plus placebo, 206 prednisolone plus valaciclovir and 206 placebo plus placebo. Outcome was measured using the Sunnybrook facial grading system (SFGS) and House-Brackmann scale (HBS) at baseline and each follow-up. Follow-ups were scheduled for day 11–17 and 1, 2, 3, 6 and 12 months after palsy onset. Significantly better outcome was seen in patients treated with prednisolone (72%) compared with patients who did not receive prednisolone (57%) (P<0.0001). Time to complete recovery was also significantly shorter in patients treated with prednisolone compared with patients not treated with prednisolone (P<0.0001). Synkinesis at 12 months was less frequent in patients treated with prednisolone (14%) compared with patients not receiving prednisolone (29%) (P<0.0001). Where treatment started within 48 hours, patients in the prednisolone group had shorter time to complete recovery, higher complete recovery rates and less synkinesis compared with the group not treated with prednisolone. Patients aged ≥40 years had significantly higher complete recovery rates if treated with prednisolone. This was not seen in patients <40 years, but synkinesis was less prevalent in patients <40 years old given prednisolone. All patients, regardless of severity at baseline, showed significantly higher complete recovery rates if treated with prednisolone compared with no prednisolone. In patients with moderate and mild palsy at baseline, significantly fewer prednisolone-treated patients had synkinesis at 12 months. Valaciclovir alone showed no effect on recovery rates, time to recovery or synkinesis and did not show any additive effect to prednisolone. In conclusion, prednisolone treatment in Bell’s palsy is recommended for use in adult patients regardless of severity of palsy and age, and should be started as early as possible, as long as no contraindications for steroid treatment is present

Fragile bones in patients with stroke? : bone mineral density in acute stroke patients and changes during one year of follow up

This thesis highlights several clinically important questions related to bone loss the first year following stroke and with regard to bone mass at stroke onset: Lack of mobility and weight bearing early after stroke is an important factor for the greater bone loss in the proximal femur on the paretic side. Relearning to walk within the first two month after stroke, even with support of another person may, however, reduce the bone loss after immobilisation. The reduction in BMD in the femoral neck appears mainly to occur in the lower part of the neck and on the paretic side. It depends on when or if the patients start to walk after stroke, but also on the amount of body weight born through the paretic leg. Thus, measuring the lower part of the femoral neck may give a better estimate of the impact of gait and weight bearing than measuring the total femoral neck. During the first year after stroke bone mineral is lost in the proximal humerus of the paretic arm, but the loss depends on the initial degree of the paresis. However, stroke patients who regain almost normal arm function within one year, despite being severely impaired initially, loose less bone mineral than patients where a severe paresis persists. Female, but not male, stroke patients have lower femoral neck BMD than population controls. At present it is unclear if low BMD actually increases the risk of stroke in women or reflects a poor health with both high stroke risk and low BMD