Left Facial Palsy Research Articles

A rare presentation of Pediatric Autoimmune Neuro-psychiatric Disorder Associated with Group A Streptococcal infections (PANDAS)

A 9 year old girl presented to us with a history of sudden onset left sided hemiparesis. Neurological examination revealed an upper motor neuron type of left facial nerve palsy, episodes of dystonia involving left upper and lower limbs and an extensor plantar response on the left side. The girl showed several adventitious movements such as choreiform movements involving the whole of the left upper extremity and the distal left lower extremity. Additionally, facial tics causing a grimacing facies were seen off and on. Interestingly, a mitral regurgitation murmur was discovered on routine clinical examinations and confirmed by echocardiography. With this background and a past history of acute tonsillitis 3 years back, we got two Anti-Streptolysin O (ASO) titres done 3 weeks apart which were markedly elevated and showed a rising titre; 406 IU/L and 815 IU/L respectively. The girl was started on oral prednisolone ,oral penicillin and oral haloperidol and she responded dramatically within 5 days. Stroke syndrome, as a cause of the acute hemiparesis, was ruled out by neuroimaging (MR-angiography). The clinical profile of our case fulfilled all the diagnostic criteria for Pediatric Autoimmune Neuro Psychiatric Disorder Associated with Streptococcal infections (PANDAS). But here, we are reporting not just another case of PANDAS, but one with a unique difference: the association of complete hemiparesis with PANDAS.

Rhino-Orbito-Cerebral Mucormycosis in a Diabetic Patient with Idiopathic CD4 + Lymphocytopenia

A 54-year-old diabetic male, with idiopathic CD4 + lymphocytopenia, was presented with a two-month history of headache and periocular pain followed by vertigo, left hypoacusia and left peripheral facial palsy and hypoesthesia. More than a month after admission, a palate ulcer appeared and Infectious Diseases consultation was required. Mucormycosis was suspected and the diagnosis was confirmed by histologic examination. Despite early surgery and combination antifungal therapy, the patient did not survive. This case report illustrates the difficulty in diagnosing a rare condition with non-specific clinical manifestations and underlines the importance of a timely multidisciplinary approach in order to recognise this highly fatal disease earlier. It also describes a previously non-reported situation of mucormycosis in a patient with idiopathic CD4 + lymphocytopenia.

Infantile osteopetrosis, craniosynostosis, and Chiari malformation type I with novel OSTEM1 mutation

We report a case of a 9-month-old Arab infant, with novel OSTEM mutation and unpublished triad of osteopetrosis (OP), craniosynostosis (CS), and Chiari malformation type I (CM1). The index presented with progressive irritability, abnormal movements, following an accidental fall. The history revealed early onset of irritability, progressive visual loss, and global developmental delay, more prominent at the gross motor level and a suspected congenital cytomegalovirus infection. The pregnancy was uneventful with subsequent unremarkable delivery. The parents are Arabs′first cousins with no apparent symptoms or signs of bone disease. Three dimensional brain computed tomography (CT) showed ventriculomegaly, thick calvaria, and CS of the coronal and sagittal sutures. Patient had signs of left lower motor neuron facial palsy, and CT of petrous bones confirms the presence of osteopetrotic petrous with slim mastoid portions of the facial nerve canals both sides. Brain magnetic resonance imaging showed CM1. Skeletal survey showed sclerotic skeleton. He needed ventriculoperitoneal shunt and died at 18 months of age. Molecular testing for OSTEM1 gene revealed novel homozygous mutation that segregated from his parents. This novel OSTEM1 gene novel mutation and the combination of OP, infantile CS, and CM1 is to our knowledge never been reported.

Holmes Tremor After Brainstem Hemorrhage, Treated With Levodopa

Holmes tremor is a rare movement phenomenon, with atypical low-frequency tremor at rest and when changing postures, often related to brainstem pathology. We report a 70-year-old female patient who was presented with dystonic head and upper limb tremor after brainstem hemorrhage. The patient had experienced a sudden onset of left hemiparesis and right facial paralysis. Brain magnetic resonance imaging showed an acute hemorrhage from the brachium pontis through the dorsal midbrain on the right side. Several months later, the patient developed resting tremor of the head and left arm, which was exacerbated by a sitting posture and intentional movement. The tremor showed a regular low-frequency (1-2 Hz) for the bilateral sternocleidomastoid and cervical paraspinal muscles at rest. The patient's symptoms did not respond to propranolol or clonazepam, but gradually improved with levodopa administration. Although various remedies were attempted, overall, the results were poor. We suggest that levodopa might be a useful remedy for Holmes tremor. The curative or relieving effect of the dopaminergic agent in Holmes tremor needs more research.

A Case of Gastric Cancer Manifesting as a Solitary Brain Metastasis in the Cerebellopontine Angle That Mimicked Acoustic Neuroma

At the time of diagnosis, about 20% of patients with gastric cancer have stage IV disease involving the liver, lung, and bone. Brain metastasis from gastric cancer is exceedingly rare, with an incidence of <1% of clinical cases. A 59-year-old man was admitted with hearing loss in the left ear and left facial palsy for 1 month. A magnetic resonance imaging scan revealed a tumor in the cerebellopontine angle that extended to the inner auditory canal and that was clinically diagnosed as acoustic neuroma. After complete resection, histological examination showed metastatic poorly differentiated carcinoma. Further investigation revealed advanced gastric cancer involving the antrum with no evidence of the involvement of other sites except the brain parenchyma. Palliative total gastrectomy was performed and the surgical specimen revealed a poorly cohesive carcinoma that was histopathologically identical to that of the resected brain tumor. Here we report this rare case of gastric cancer that initially presented as a solitary brain metastasis mimicking acoustic neuroma.

感染症内科外来で診断に 1 年以上を要したライム病の 1 例

A 42-year-old woman presenting with years of fever and vague symptoms could not be satisfactorily diagnosed in physical examination or conventional workups. She was presumptively diagnosed with chronic fatigue syndrome and treated symptomatically. Fourteen months after the initial visit, she developed left facial palsy. Lyme disease serology was positive. Four weeks of oral amoxicillin ameliorated symptoms. Only 5 to 15 cases of Lyme disease are reported annually in Japan, mostly from the northeastern-most island of Hokkaido. It may occur anywhere in Japan, however; probably is underdiagnosed. Lyme disease may cause fevers of unknown origin. Astute clinical suspicion and appropriate workups are thus needed to diagnose this infection.

A Minor Modification of Direct Browplasty Technique in a Patient with Brow Ptosis Secondary to Facial Paralysis: Copy-Paste-Excise and Stitch

Purpose. This report aimed to describe a minor modification of the traditional direct browplasty technique that aids in surgical planning for patients with brow ptosis secondary to facial paralysis without changing the shape of the brow. Case Report. A 74-year-old male patient with left facial paralysis secondary to chronic otitis media was referred with a complaint of low vision due to brow ptosis. We performed direct browplasty with a minor modification in order to aid a treatment customized to the patient. In this technique, a transparent film paper is used to copy the brow shape. A brow-shaped excision is facilitated just superior to the ptotic brow. Conclusion. The authors found that the copy-paste-excise and stitch technique was effective and successful for deciding the shape and the amount of excision that should be performed in patients with brow ptosis without resulting in asymmetrical, arched, and feminized brows.

Image Diagnosis: Ramsay Hunt Syndrome

A 68-year-old woman presented to the Emergency Department with left facial weakness (Figure 1). She reported upper and lower motor facial weakness. She was diagnosed with left-sided Bell's palsy and treated with steroids. She returned two days later with chief complaint of left ear pain and swelling. She denied having fever or hearing loss. Ramsay Hunt syndrome (Herpes zoster oticus) by strict definition is a peripheral facial nerve palsy accompanied by an erythematous vesicular rash on the ear (zoster oticus) or in the mouth. The triad of ipsilateral facial paralysis, ear pain, and vesicles in the auditory canal and auricle is typically present (Figure 2).1 Various clinical presentations of facial paralysis and rash such as such tinnitus, hearing loss, nausea, vomiting, vertigo, and nystagmus can be present. This has been attributed to close proximity of the geniculation ganglion to the vestibulocochlear nerve within the bony facial canal. In a prospective study of patients with Ramsay Hunt syndrome, 14% developed vesicles after the onset of facial weakness. Thus, Ramsay Hunt syndrome may initially be indistinguishable from Bell's palsy.2 The facial paralysis seen in Ramsay Hunt syndrome is typically more severe than Bell's palsy, attributed to herpes simplex virus with a decreased probability of complete recovery.3 Retrospective studies have shown earlier administration of steroids along with antivirals within 3 days of symptom onset have 75% rate of full recovery versus only 30% if combined therapy is started 7 days after onset of symptoms. The typical combined therapy involves a 7- to 10-day course of famciclovir (500 mg, 3 times daily) or acyclovir (800 mg, 5 times daily), along with oral prednisone (60 mg daily for 3 to 5 days).4 Figure 1 (left) Left upper and lower motor facial paralysis. Figure 2 (above) Cutaneous erythematous vesicular eruption and swelling of ipsilateral left ear.

Guillain-Barré syndrome following severe head trauma and spine surgery

Guillain-Barré syndrome (GBS) is an acute-onset inflammatory polyradiculoneuropathy usually triggered by an infectious disease. In some cases, GBS can occur without any preceding infectious episode, like after vaccination, epidural anaesthesia or surgery. A 73 years old woman had head and spine trauma. Body-TDM showed bilateral temporal and right frontal haematomas and fracture of the first lumbar vertebrae. Sextant and kyphoplasty were performed. She presented 14 days after surgery tetraparesis, swallowing difficulties and bilateral facial palsy. Electromyography was consistent with demyelinating neuropathy. Cerebrospinal fluid examination found albumino-cytological dissociation. Viral and bacterial serology and antiganglioside antibodies were negative. She was treated with intravenous immunoglobulins. Four months after discharge she had fully recovered except left peripheral facial palsy. GBS can rarely be triggered by head trauma or spine surgery. Physician must keep in mind this diagnosis whenever their patients present acute-onset neurological worsening in such context.

Unilateral lower motor neuron-type of facial palsy following snake bite presumably due to Krait (Bungarus Caeruleus)

We describe an unusual case of snake bite presumably due to Krait (Bungarus Caeruleus) in a 12-year-old child from Mohali, Punjab. He presented with a history of bite behind his left ear, while he was sleeping at night on floor. He had bilateral ptosis, dysphonia initially, which progressed gradually to cause respiratory paralysis. Child was managed with antisnake venom, ventilation and other supportive measures. He recovered gradually, but persisted to have lower motor neuron paralysis of left facial nerve, which was noted post extubation. This uncommon presentation could be because of exposure of the facial muscles to the venom, spreading directly from the injection site and destroying the nerve terminals of facial nerve in the muscle tissue. At three months follow-up, child showed complete recovery. Facial nerve involvement following snake bite, which usually has a good prognosis, remain an uncommon presentation in paediatric age group. J Nepal Paediatr Soc 2012;32(2):184-186 doi: http://dx.doi.org/10.3126/jnps.v32i2.5824

Renal Involvement in Antimyeloperoxidase Antineutrophil Cytoplasmic Antibody-positive Granulomatosis with Polyangiitis with Chronic Hypertrophic Pachymeningitis

To the Editor: Granulomatosis with polyangiitis (GPA; formerly Wegener’s granulomatosis) is a small to medium-size vessel vasculitis involving various organs, with the classic triad of upper airway, lung, and kidney involvement. GPA can be accompanied by chronic hypertrophic pachymeningitis (CHP), characterized by inflammatory thickening of the cerebral or spinal dura mater1. Although the pathology of CHP does not depend on the contiguous spread of granulomatous lesions from the nasal or paranasal sinuses, GPA with CHP has been reported to present mostly with limited organ involvement2,3. Specifically, antimyeloperoxidase antineutrophil cytoplasmic antibody (MPO-ANCA)-positive GPA with CHP has been reported to lack renal involvement1,2,4. We describe a case of MPO-ANCA-positive GPA, which presented with CHP-related central nervous system (CNS) symptoms and with renal abnormalities, with a discussion of the incidence of renal involvement in MPO-ANCA-positive GPA accompanied by CHP. A previously healthy 48-year-old Japanese man presented to the otolaryngology department with a 3-month history of fever, bilateral temporal headaches, left facial nerve paralysis, dysphagia, and hoarseness, which occurred sequentially. … Address correspondence to Dr. H. Kohsaka, Department of Medicine and Rheumatology, Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University, 1-5-45 Yushima Bunkyo-ku, Tokyo 113-8519, Japan. E-mail: kohsaka.rheu{at}tmd.ac.jp

513 Heerfordt´S Syndrom in an Adolescent Boy

IntroductionHeerfordt's Syndrome is characterized by bilateral uveitis, facial paralysis, fever and parotitis which is a rarely seen condition in sarcoidosis with neurological manifestations.Case reportA fifteen year old boy admitted to...

Neurological symptoms following exposure to ozone

The proven efficacy and safety of medical ozone therapy remains controversial [1–6]. In rats, ozone inhalation was shown to promote neuronal activation in stress-responsive regions of the central nervous system (CNS) [7], electrical activity alterations and changes in brain major neurotransmitter systems [8, 9]. We here describe two patients who developed cortical blindness and seizures shortly after intraarticular ozone infiltrations. There is only a prior report of top of the basilar stroke and Anton syndrome following ozone therapy [10]. A 75-year-old man complained of dizziness, slurred speech, tingling in the left extremities and visual loss, which developed 10 min after receiving a paravertebral ozone therapy session and autohemotherapy for spondylarthrosis. On admission he was fully aware with left neglect, cortical blindness, left central facial paralysis, and numbness and mild weakness of the left extremities. A brain magnetic resonance imaging (MRI) scan showed subcortical/juxtacortical white matter chronic ischemic lesions in the right cerebral hemisphere without evidence of acute lesions (Fig. 1a). Stenosis of the distal branch of the left cerebral posterior artery and a severe stenosis of the right carotid artery on MR angiograms were found. An EEG showed an alpha rhythm (8 Hz) predominantly in the left posterior region and a slow attenuated delta rhythm in the right hemisphere. A Tc-ECD brain SPECT showed bilateral hypoperfusion areas particularly in the left occipitotemporal region (Fig. 2). Twenty-four hours later, the patient presented visual hallucinations, various episodes of forced oculocephalic deviation to the left, clonus on left extremities and a tonic–clonic seizure. He was successfully treated with valproic acid and levetiracetam. A brain MRI performed 7 days later showed one small juxtacortical subacute ischemic lesion in the right occipital lobe (Fig. 1b). A 60-year-old woman presented with posterior cortical blindness and history of a tonic–clonic seizure, which developed shortly after intraarticular infiltration of ozone and procaine for the treatment of a left shoulder joint disease. On admission she was disoriented with repetitive speech, anterograde memory impairment and cortical blindness. The brain MRI showed a right acute ischemic cerebellar lesion (Fig. 1c). The EEG showed slowed brain D. V. Rolan (&) J. L. B. Cunat J. V. Hervas D. Escudero Neuroscience Department, Hospital Universitari Germans Trias i Pujol, Universitat Autonoma de Barcelona, Carretera de Canyet s/n, 080916 Badalona, Barcelona, Spain e-mail: doloviro@hotmail.com

Paclitaxel‐based chemotherapy for aggressive kaposiform hemangioendothelioma of the temporomastoid region: Case report and review of the literature

Kaposiform hemangioendothelioma (KHE) is a rare vascular tumor of infancy and childhood. This tumor results in poor prognosis, and therefore, development of a more effective treatment is needed. We describe an 11-year-old boy presenting with left facial palsy caused by aggressive KHE of the left temporomastoid region. He was treated with paclitaxel-based chemotherapy, because of the difficulty with complete surgical resection for anatomic factor, multiple lung metastases on diagnosis, and no response to conventional treatments. This treatment reduced the volume of primary tumor and lung metastatic lesions, but the efficacy was transitory. Paclitaxel-based chemotherapy for aggressive KHE may be effective, therefore the multimodality therapy including paclitaxel of aggressive KHE, particularly in the head and neck, needs to be investigated in further studies.

Case report: airway and concurrent hemodynamic management in a neonate with oculo‐auriculo‐vertebral (Goldenhar) syndrome, severe cervical scoliosis, interrupted aortic arch, multiple ventricular septal defects, and an unstable cervical spine

We report the challenging case of a 1-week-old, term, 2.4 kg neonate with Goldenhar syndrome (including microcephaly, left microtia, left facial palsy, dextro-scoliosis of the cervical spine, and cervico-thoracic levoscoliosis), multiple ventricular septal defects, a type B interrupted aortic arch, a large patent ductus arteriosis, and radiographic and clinical signs concerning for an unstable cervical spine. Our anesthesia team was consulted for perioperative management of this patient during her surgical repair. This case report describes the use of the Air-Q size 1 laryngeal airway (LA) to assist fiberoptic intubation in an ASA 4 neonate with cardiac disease, an anticipated difficult airway with the addition of an unstable cervical spine, as well as the anesthetic techniques used to maintain hemodynamic stability while the airway was secured.

Facial paralysis associated to hypothyroidism in a dog

The hypothyroidism is the most commonly endocrinopathy in dogs, that occurs preferentially in middle-aged pure breed. The clinical signs associated with hypothyroidism are variable, many times non-specific, including metabolical, dermatological or cardiovascular. The main laboratorial findings are non-regenerative anemia and hypercholesterolemia. Hyponatremia, increase on alanine transferase and alkaline phosphatase activity also can be observed in a lower frequency. There are some reports of peripheral neuropathies caused by hypothyroidism, but the pathophysiology of this process is still unknown. There are specific diagnostic tests that can be used to help diagnose hypothyroidism, and those should be aligned together with the animal´s clinical symptoms. The thyroxine stimulating hormone, and free and total thyroxine concentrations are the most used tests. A Pit Bull dog, female, over weighted, was treated presenting left facial paralysis. Thyroid function tests confirmed hypothyroidism. The animal was treated with hormonal replacement and there was improvement in clinical signs in 40 days, confirming that hypothyroidism was facial paralysis’ cause.

English

Stenotrophomonas maltophilia is a gram negative bacterium that primarily affects immunocompromised and clinically debilitated patients. It is known to affect many tissues and organ systems of the body, but it rarely causes otitis externa. We reported a case of otitis externa due to S. maltophilia in a 22-year-old immunocompromised male who had bilateral otalgia and ear discharge, as well as left facial nerve palsy in combination with hearing loss. Further investigations revealed S. maltophilia in ear drainage cultures, including a deep swab taken during surgical debridement. Systemic antibiotics in combination with local treatment were effective in controlling the infection and resulted in the resolution of his signs and symptoms, except for the hearing loss. We believe that early identification of the pathogen and treatment of the infection might lead to a favorable outcome. Key words: Stenotrophomonas maltophilia, opportunistic infection, otitis externa.

Hemiplegic peripheral neuropathy accompanied with multiple cranial nerve palsy.

A 32-year-old man experienced double vision around January, 2010, followed by weakness of his left upper and lower extremities. Articulation disorders and loss of hearing in his left ear developed, and he was admitted to our hospital on February 14, 2010. Physical examination was normal, and neurological examination showed clear consciousness with no impairment of cognitive function, but with articulation disorders. Olfactory sensation was reduced. Left ptosis and left gaze palsy, complete left facial palsy, perceptive deafness of the left ear, and muscle weakness of the left trapezius muscle were observed. Paresis in the left upper and lower extremities was graded 4/5 through manual muscle testing. Sensory system evaluation revealed complete left-side palsy, including the face. Deep tendon reflexes were slightly diminished equally on both sides; no pathologic reflex was seen. No abnormality of the brain parenchyma, cerebral nerves or cervicothoracolumbar region was found on brain magnetic resonance imaging. On electroencephalogram, alpha waves in the main frequency band of 8 to 9 Hz were recorded, indicating normal findings. Brain single photon emission computed tomography (SPECT) scan showed reduced blood flow in the right inner frontal lobe and both occipital lobes. Nerve biopsy (left sural nerve) showed reduction of nerve density by 30%, with demyelination. The patient also showed manifestations of multiple cranial nerve disorder, i.e., of the trigeminal nerve, glossopharyngeal nerve, vagus nerve, and hypoglos-sal nerve. Whole-body examination was negative. Finally, based on ischemic brain SPECT images, spinal fluid findings and nerve biopsy results, peripheral neuropathy accompanied with multiple cranial nerve palsy was diagnosed.

A patient with Marchiafava–Bignami disease as a complication of diabetes mellitus treated effectively with cortico steroid

We report a 37-year-old woman with no history of alcohol consumption or malnutrition who had Marchiafava–Bignami disease (MBD) as a complication of diabetes mellitus. The patient suddenly developed dizziness and could not speak words fluently. Neurological examination revealed acalculia, agraphia, left blepharoptosis, and mild left facial palsy. Her blood glucose was 391mg/dL, and her glycated hemoglobin (HbA1c) was 16.0%. Her brain MRI revealed hyperintense changes in the corpus callosum on T2-weighted, fluid-attenuated inversion recovery and diffusion-weighted images. In addition to therapy for diabetes mellitus, prednisolone was commenced. All of the symptoms gradually improved, and after three months, only slight dysarthria, acalculia, and agraphia were observed. Brain MRI also revealed a reduction in lesion size. In conclusion, MBD may occur even with metabolic disorders. It is important to diagnose MBD in the early stages with MRI and to treat the symptoms with cortico steroids.

Otitis externa due to Stenotrophomonas maltophilia in an immunocompetent patient: Case report

Stenotrophomonas maltophilia is a gram negative bacterium that primarily affects immunocompromised and clinically debilitated patients. It is known to affect many tissues and organ systems of the body, but it rarely causes otitis externa. We reported a case of otitis externa due to S. maltophilia in a 22-year-old immunocompromised male who had bilateral otalgia and ear discharge, as well as left facial nerve palsy in combination with hearing loss. Further investigations revealed S. maltophilia in ear drainage cultures, including a deep swab taken during surgical debridement. Systemic antibiotics in combination with local treatment were effective in controlling the infection and resulted in the resolution of his signs and symptoms, except for the hearing loss. We believe that early identification of the pathogen and treatment of the infection might lead to a favorable outcome. Key words: Stenotrophomonas maltophilia, opportunistic infection, otitis externa.