A case of Ramsey Hunt syndrome with multiple cranial nerve paralysis and acute respiratory failure]

The authors report a 56-year-old woman with Ramsey Hunt syndrome with multiple cranial nerve paralysis and acute respiratory failure. Five days before admission, she experienced right otalgia and right facial pain and consulted an otolaryngologist of our hospital, who diagnosed the illness as acute parotitis and laryngopharyngitis. One day before admission, she experienced mild dyspnea and general fatigue and came to our hospital emergency room. A chest X-ray film revealed no abnormalities but some blisters were observed around her right ear. The next day, her dyspnea became more severe and she was admitted. A chest X-ray film on admission revealed right lower lobe consolidation, and neurological examination disclosed multiple cranial nerve paralysis, i.e., paralysis of the right fifth, seventh, eighth, ninth, tenth, eleventh, twelfth and left tenth cranial nerve. The serum titer of anti-herpes zoster antibody was elevated to 1,024, and the patient was diagnosed as having Ramsey Hunt syndrome with multiple cranial nerve paralysis. Arterial blood gas analysis revealed hypoxemia with hypercapnea, which was considered to be due to aspiration pneumonia and central airway obstruction caused by vocal cord paralysis. Mechanical ventilation was soon instituted and several antibiotics and acyclovir were administered intravenously, with marked effects. Three months after admission, the patient was discharged with no sequelae except mild hoarseness. Patients with herpes zoster oticus, facial nerve paralysis and auditory symptoms are diagnosed as having Ramsey Hunt syndrome. This case was complicated by lower cranial nerve paralysis and acute respiratory failure, which is very rare.(ABSTRACT TRUNCATED AT 250 WORDS)     

Intramedullary spinal cord metastasis of lung adenocarcinoma causing Brown-Séquard Syndrome]

A 36-year-old woman admitted to our hospital because of numbness in the left limbs and weakness in the right arm, and was subsequently given a diagnosis of intramedullary spinal cord metastases from lung cancer. The patient had lung adenocarcinoma with metastases to the brain, spine and lymph nodes. Occipital craniotomy, radiation therapy and chemotherapy were performed on the lesions in the year following June 1994. In June 1995, however, she complained of numbness in the left limbs and weakness in the right arm. Compatible with her neurological manifestation, MRI demonstrated tumors in the right side of the cord at the spinal level of C3-4 and C7-Th1, both of which were of high density in T2-enhanced conditions with enhancement by gadolinium-diethylenetriamine pentaacetic acid. No invasion from spinal metastasis was detected by CT, scintigraphy or MRI. We therefore diagnosed her manifestation as Brown-Séquard syndrome caused by intramedullary spinal cord metastatic tumors of lung adenocarcinoma. In order to avoid paraplegia and dysfunction of the bladder and bowel, radiation therapy of the cord lesions with total dose of 44 Gy was performed. Her neurologic manifestation was improved, restoring her quality of life, as the tumor size estimated by MRI decreased. Four months later, however, she died of lung adenocarcinoma that developed accompanied with severe peritonitis carcinomatosa and multiple metastases.     

Spinal cord sarcoidosis without abnormal shadows on chest radiography or chest CT diagnosed by transbronchial lung biopsy]

The patient was a woman aged 56 years. In February 1998, she complained of fatigability of the right upper limb and disturbed extension of the right fourth finger. Because her condition deteriorated gradually and myelopathic signs such as difficulty in walking developed, she was hospitalized in May 1999 for close examination and appropriate treatment; and she was a few days later transferred to our hospital because of progressive myelopathy. In T2-weighted MR images of the cervical spine, the high-intensity area ranged between C2 and Th1, and in Gd-DTPA enhanced MRI the high-intensity area was seen between C3 and C7. Although chest radiographs and chest CT scans were normal, spinal cord sarcoidosis could not be ruled out, and therefore, bronchoscopic examination was performed. Specimens obtained from transbronchial lung biopsy (TBLB) revealed non-caseating epithelioid cell granulomas, and so and the disease was diagnosed as spinal cord sarcoidosis. Both symptoms and MRI findings were improved by treatment with corticosteroids. It is suggested that, in patients suspected of spinal cord sarcoidosis from MRI findings. TBLB should be aggressively attempted, even if chest radiographs and chest CT scans are normal.     

Primary central nervous system lymphoma masquerading as bilateral vitreous floaters

A 72-year-old female presented with a 6-month history of bilateral floaters and visual blurring. Clinically, the posterior vitreous was cellular bilaterally, with no signs of subretinal infiltrates, retinal vasculitis, disc oedema or macula oedema. A vitreous biopsy and vitrectomy were scheduled following left cataract surgery because of the presence of a dense cataract. One month after cataract surgery, the patient developed signs of florid left arteritis involving the first-order branches of the central retinal artery. A 23-gauge vitreous biopsy and vitrectomy were performed, and preservative-free triamcinolone was injected. Cytology of the biopsy demonstrated benign T-lymphocytes and histiocytes suggestive of mild chronic inflammation only. Magnetic resonance imaging (MRI) of the brain was normal as was lumbar puncture. Subsequently, the patient developed right upper motor neuron facial nerve palsy. MRI imaging on this occasion demonstrated multiple hyper-intense white matter lesions. A third MRI was subsequently obtained due to new neurological deficits and demonstrated enlargement of the pre-existing lesions. Brain biopsy confirmed the presence of primary cerebral lymphoma. The present case highlights the role of various tissue biopsies, including vitreous, cerebrospinal fluid and brain tissue, to establish an elusive diagnosis of primary central nervous system lymphoma presenting as benign vitreous floaters.     

Improvement of cerebral perfusion with argatroban in a patient with antiphospholipid syndrome]

A 22-year old woman developed erythema in her hands. She also suffered from general fatigue and arthralgia. In July of 1992, she visited the dermatology clinic of Nagoya City University Hospital. We made a diagnosis of subacute cutaneous lupus erythematosus with thrombocytopenia and gave her 30 mg of prednisolone daily. Her conditions were improved and had been stable with 5 mg of prednisolone daily. In the winter of 1993, she developed ulcer in her fingers and soles. These lesions were associated with antiphosholipid syndrome. She complained of mild headache. She was examined by CT, MRI and single photon emission computerized tomography (SPECT) using N-isopropyl-p-[123I] iodoamphetamine (IMP). Low perfusion areas in the bilateral occipital lobes and the right temporal lobe were found in SPECT, although no abnormalities were detected by CT and MRI. Low perfusion areas in antiphospholipid syndrome might be caused by microarterial thrombosis, microvenous thrombosis or spasms, although they could be reversible. Ten mg of argatroban daily for one month improved cerebral low perfusion, headache, and skin lesions. In this case, argatroban was effective for both cerebral low perfusion and skin ulcer, which were related to antiphospholipid syndrome.     

Choreic involuntary movement that occurred during therapy for diabetes mellitus

A 73-year-old woman was admitted with dry mouth, polyposia, polyuria, hyperglycemia (611 mg/dl) and positive urine ketone bodies. Blood glucose levels decreased gradually after initiation of insulin injections. The patient was discharged, but developed involuntary movement of the right extremities on the following day. At that time, her blood glucose levels were 54 mg/dl. Four days later, she was admitted to our Neurology Ward because the movement worsened. On admission, choreic involuntary movements were severe in the right extremities and slight in the left extremities. Urine ketone bodies were negative, but HbA1c had elevated to 11.7%. Although a brain CT did not detect any abnormal density areas, we suspected that the patient had cerebral infarction of the basal ganglia or the parietal lobe on the left side, or of the subthalamic nucleus on the right side because choreic involuntary movements were more prominent on the right side. Anti-platelet therapy was performed, but the involuntary movements persisted. A T1-weighted image of brain MRI, performed on the 4th day after hospitalization, detected abnormal high intensity areas in the bilateral putamens. The abnormal area in the left putamen was more prominent. This MRI finding was consistent with that of diabetic chorea-ballism. Surface electromyography demonstrated the simultaneous appearance of grouping discharges in the biceps and triceps muscles of the right arm. We diagnosed the patient as having diabetic chorea based on the MRI findings, and discontinued anti-platelet therapy. MRI should be performed as soon as possible in diabetic patients with acute-onset chorea-ballism which occurs on one side or predominantly on one side in order to differentiate diabetic chorea-ballism from cerebral infarction.     

A case of elderly onset loss of consciousness due to bacterial meningitis and subsequent vasculitis

We reported an 83-year-old woman, who suffered from bacterial meningitis and subsequent vasculitis. She experienced episodes of loss of consciousness several times in July, 2006. She also had recurrent fever and was admitted to a local hospital. Routine examinations, including brain MRI and electroencephalogram, were negative and urinary tract infection was diagnosed. After successful antibiotic therapy, she was transferred to a rehabilitation hospital. After transfer, she had no headache, but presented fever again, and a reduced level of consciousness. Cerebrospinal fluid test showed that cell counts were high with a predominance of neutrophils, and her glucose level was low. She received antibiotic therapy on her suspicion of bacterial meningitis. Bacterial cultures of CSF and blood were negative, probably due to the previous antibiotic therapy. Repeated CSF analysis showed a decrease in cell counts, but her lower consciousness did not improve. Moreover, neurological symptoms such as left pyramidal tract sign appeared. She was transferred to our hospital on the suspicion of vasculitis. Diffusion MRI showed high intensity in the right middle cerebral artery (MCA) area and CT angiography showed the stenosis of the right MCA at the M3 portion. Two courses of steroid pulse therapy were performed. Her consciousness gradually improved and eventually could talk although cognitive decline remained as a residual deficit. Our patient failed to be diagnosed early because of atypical symptoms of meningitis. Caution seems necessary for elderly cases presenting with atypical initial symptoms of meningitis. Steroid pulse therapy was effective for the subsequent vasculitis, as reported previously.     

Primary alveolar hypoventilation syndrome complicated with antiphospholipid syndrome

A 32-year-old woman was transported to our hospital by ambulance because of loss of consciousness and breathing induced by drug intoxication. After general status was recovered, her arterial blood gas analysis under breathing room air revealed hypercapnia and hypoxemia which were caused by hypoventilation. After exclusion of apparent pulmonary, neuromuscular and central nerve diseases, she was diagnosed with primary alveolar hypoventilation syndrome. She had the complication of antiphospholipid syndrome (APS), suggesting the possibility of small lesions of the brainstem due to APS, which were too small to be detected on CT or MRI; these small lesions could cause injuries to the respiratory center.     

Persistent Hemichorea as a Preceding Symptom of Cerebral Infarction Due to Middle Cerebral Artery Stenosis

We herein report an 84-year-old woman with right middle cerebral artery (MCA) stenosis who presented with persistent left hemichorea preceding cerebral infarction. She visited our hospital on day 9 after the hemichorea onset. Magnetic resonance imaging (MRI) showed no acute cerebral infarction. Magnetic resonance angiography revealed right MCA stenosis. Her hemichorea persisted for 19 days and subsequently disappeared. On day 21, she developed left hemiplegia. Repeat MRI revealed a cerebral infarction in the right putamen. MCA stenosis can present with persistent hemichorea, even in the absence of cerebral infarction. Persistent hemichorea with MCA stenosis may presage cerebral infarction.     

Brain tumor-like lesion in Behçet disease

We report a patient with longstanding Behçet disease who presented sudden onset of headache and facial paresis. The magnetic resonance imaging (MRI) showed a mass in the right thalamus, extending to the lentiform nucleus, subthalamic area, right cerebral peduncle and deep subcortical white matter. Stereotactic brain biopsy disclosed gliosis with no signs of malignancy. The diagnosis of a pseudotumoral form of neuro-Behçet disease was done and she was treated with pulse methylpredinisolone and intravenous cyclophosphamide. After 8 weeks she had improved and a new MRI showed disappearance of the tumor-like lesion. The differential diagnosis, especially with central nervous system tumor is emphasized.     

Psychomotor seizures

A 30-year-old woman presented to the emergency department after an episode of "dizziness" that caused her to lose control of her car. During the preceding 12-month period she had had recurrent "dizzy" spells and abdominal pains for which she was evaluated by several physicians in various emergency departments. Physical and general neurological examination on presentation was within normal limits. Outpatient CT scan was normal, and an EEG revealed a right temporal lobe spike with slow wave pattern suggestive of temporal lobe focus. Since begun on therapeutic doses (300 mg per day of phenytoin), the patient has not experienced further spells or abdominal pains. This report emphasizes the need to be cognizant of an underlying seizure disorder as a possible etiologic agent responsible for a traumatic incident.     

Analysis of systemic lupus erythematosus (SLE) involving the central nervous system by magnetic resonance imaging (MRI)

Involvement of the central nervous system (CNS) commonly occurs in systemic lupus erythematosus (SLE). But definitive diagnosis remains difficult even with computed tomography (CT). In this study, we used the recently developed technique, magnetic resonance imaging (MRI) for CNS lupus and compared it with CT scans. CT was performed with a General Electric 8800 CT/T scanner. MRI was performed using a Mitsubishi Electric MMI-150 S. Ten patients with CNS lupus were divided into 3 groups. Group I included 4 cases with neurological manifestations alone. All lesions seen on CT were also detected by MRI, with greater clarity and extent. Furthermore, MRI depicted several microinfarcts in white matter without symptoms. Group II included 5 cases with psychiatric features alone. MRI detected a thalamic microinfarct in only one case while CT showed no abnormality in all cases. Group III included 1 case with both neurological and psychiatric symptoms. MRI demonstrated a small infarct of midbrain corresponding with neurological symptoms, more clearly than CT. Therefore MRI demonstrates the degree of brain involvement in SLE more accurately than CT.     

Serial CT and MRI findings in a patient with isolated angiitis of the central nervous system associated with cerebral amyloid angiopathy

We report serial CT and MRI findings in a biopsy-proven case of cerebral amyloid angiopathy (CAA) with isolated angiitis of the central nervous system (CNS). A 69-year-old man had developed dizziness, dementia, and generalized seizure during the preceding 4 years. An initial examination by brain CT and MRI showed bilateral symmetrical periventricular lesions closely resembling those of Binswanger's disease. Subsequently, the lesions expanded slowly, involving a large area of the right cerebral hemisphere with an obvious mass effect. Since a primary brain tumor was suspected, a brain biopsy was performed, and histopathological examination revealed amyloid beta protein CAA within the meningocortical vessels associated with perivascular monocytic cuffing, indicating the presence of isolated angiitis of the CNS. Multinucleated giant cells containing intracytoplasmic beta protein amyloid around a heavily amyloid-laden cortical vessel were also observed. This is the first case report to show sequential radiographical studies of the leukoencephalopathy associated with CAA and isolated angiitis of the CNS.     

Clinical and Radiological Profiles of COVID-19 Patients with Neurological Symptomatology: A Comparative Study

Patients with COVID-19 can require radiological examination, with chest CT being more frequent than neuro-imaging. The objective is to identify epidemiological, clinical and radiological factors considered as predictors of neurological involvement in patients with COVID-19 assessed by neuroimaging and to describe the neuroimaging findings. This retrospective study was performed with 232 consecutive confirmed COVID-19 patients, from two radiological units, which were divided into two groups: (1) those who underwent a brain CT/MRI scan (n = 35) versus (2) those who did not undergo the brain CT/MRI scan, but underwent only chest CT (n = 197). There was a statistically significant difference with associations regarding the COVID-19 brain scan group for: admission to ICU, greater severity of lung injuries, the use of a mechanical ventilator and sepsis. Statistical tendency was found for chronic renal failure and systemic arterial hypertension. Forty-percent of COVID-19 patients from the brain scan group were abnormal on brain CT and/or brain MRI (22.9% of the cases with bleeding or microbleeding, 8.6% with restricted diffusion lesions). One ischemic stroke case was associated with irregularity at the M1 segment of the right middle cerebral artery. There was a case of left facial nerve palsy with enhancement of the left geniculate ganglia. An analysis of the olfactory bulbs was possible in 12 brain MRIs and 100% had enhancement and/or microbleeding. In conclusion, a more severe COVID-19 disease from ICU, a more severe form of lung disease, the use of mechanical ventilator and sepsis were associated to the COVID-19 patients with neurological involvement who had undergone brain scans. Microvascular phenomenon was a frequent finding in the brain and olfactory bulbs evaluated by neuroimaging.     

Transient tetraparesis after intrathecal and high-dose systemic methotrexate

 Aggressive polychemotherapy, intrathecal cytostatic prophylaxis and cranial irradiation have contributed to the remarkable improvement in the prognosis of acute lymphoblastic leukemia (ALL) and subtypes of high-grade non-Hodgkin's lymphoma (NHL) and the reduction of central nervous system (CNS) relapses. Early and late neurologic changes have been observed after different CNS-directed therapies. We report on the rare event of an acute tetraparesis after methotrexate (MTX) without other CNS-directed therapy. A young female with a diffuse large B-cell lymphoma developed signs of meningeal irritation a few hours after intrathecal prophylaxis with MTX, cytosine-arabinoside and dexamethasone. She recovered quickly. Ten days after her last course of systemic chemotherapy including high dose MTX she was admitted with a tetraparesis and motoric aphasia. A computer assisted tomography (CT) scan was normal. On magnetic resonance imaging (MRI) hyperintense white matter lesions were visible in the periventricular white matter. Initially, the radiologic signs were progressive while the patient's clinical condition improved. MRI controls after complete neurologic normalization revealed delayed partial regression of the white matter abnormalities. The patient has now been free of neurologic symptoms for 16 months. This case report demonstrates acute and subacute neurotoxic effects of MTX in the same patient and illustrates that radiologic CNS changes can persist irrespective of the disappearance of clinical symptoms. Received: March 13, 1998 / Accepted: July 29, 1998     

Diabetic ketoacidosis and rhino-orbital mucormycosis

Mucormycosis often develops in immunocompromised patients, particularly in patients with diabetic ketoacidosis. Unless early diagnosis and treatment is established mucormycosis leads rapidly to death. A 38-year-old woman was admitted to the hospital with a severe diabetic ketoacidosis. Her clinical status improved in 4 days as a result of aggressive medical treatment. She has complained left cheek pain on the 10th day and had a swelling of her left cheek, facial edema, a black eschar on the palate and nasal cavity in association with visual disturbance and total ophthalmology in a short time. CT scan revealed left orbital cellulitis and pansinusitis. Excessive surgical treatment was performed and liposomal amphotericin-B, 4 mg/(kg day) was applied. Extensive fungal invasion of the orbit and the sinuses was demonstrated in the pathological species and Rhizomucor species were yielded with culture. Repeated superficial debridement was also performed. After 10 weeks, she was discharged with suggestion of insulin treatment and liposomal amphotericin-B with progressively decreasing doses. At the 13th month following the presentation, the patient was free of disease as confirmed by serial imaging and under good glycaemic control with insulin treatment. Although mucormycosis is a fatal infection, early diagnosis and aggressive treatment may decrease mortality.     

Encephalitis in a renal transplantation patient

The patient was a 55 year-old-woman with chronic renal failure due to idiopathic mesngial deposition of Ig A. She received a second allograft of a kidney from a cadaver. Results of a preoperative serologic Ig G tests for EBV and CMV were positive. She was given triple-drug immunosuppressive therapy, consisting of cyclosporine,azathioprine, and steroids. Seven years later, azathioprine was changed to mycophenolate mofetil. One year later, she was admitted to the hospital with a three to four week history of vertigo (which did not improve after sulpiride was administrated) and an influenza-like syndrome. A CT scan of the brain appeared normal, so paroxysmal positional vertigo was the diagnosis. Two weeks after admission to the hospital, the patient reported visual hallucinations and impairment of consciousness. Results of laboratory tests were leukocyte increase (polymorphonuclear leukocytes), anemia, hyponatremia and renal failure. Chest radiography, brain CT, and electroencephalography revealed no pathologic signs. The CSF examination revealed 300 cells/ml (79% PMNL), glucose 63 mg/dl, protein 45 mg/dl. Six hours later the treatment was initiated with ampicillin, ceftriaxone and ganciclovir iv, she experienced seizures that affected the left side of her body, but without interictal recovery. The patient required intubation and mechanical ventilation in the intensive care unit. An MRI of the brain images, revealed high signal-intensity regions indicating lesions on the bulb, protuberance, mesencephalon, left thalamus and parenchyma adjacent to the corpus callosum (fig. 1). Six days later, the patient partially recovered consciousness, and she had not neurologic sequelae. Intubation was terminated. As soon as PCR revealed EBV DNA in CSF samples, the treatment with ceftriaxone and ampicillin was discontinued. Treatment with ganciclovir was maintained for 8 weeks (4 weeks with iv and another 4 weeks with oral treatment). On day 35, the examination of a specimen of CSF revealed: glucose 46, protein 78, 15 cells/ml (100% lymphocytes). The patient went home on day 55 after admission to our hospital. She regained her normal neurologic function. Three weeks later MRI, showed reduction of the size of the lesions and the lesions on the brain stem had disappeared.     

Musculoskeletal ultrasound diagnosis of quadrilateral space syndrome: A case report

Introduction:Quadrilateral space syndrome (QSS) is a peripheral nerve entrapment disease, which can be misdiagnosed in clinic. In the past, QSS was mainly diagnosed by clinical symptoms combined with magnetic resonance imaging (MRI), electromyography (EMG), and arterial angiography. There are few reports on the diagnosis of QSS by musculoskeletal ultrasound (MSKUS) combined with clinical symptoms.Patient concerns:A middle-aged female patient had posterolateral pain and numbness in her right shoulder for 2 months.Diagnoses:At first, she was diagnosed as suprascapular nerve entrapment, while EMG of suprascapular nerve and axillary nerve indicated that nerve conduction was normal. Then, MRI was performed, showing the shoulder had no abnormalities, and EMG and arterial angiography of upper limb showed no abnormalities too. Finally, she was diagnosed as QSS according to MSKUS and lidocaine block test.Interventions:Two sealing treatments of axillary nerve block in quadrilateral space under the guidance of MSKUS were performed.Outcomes:After 2 treatments, the pain and numbness in her shoulder disappeared, and her shoulder could move normally. There was no recurrence after 3 months of follow-up.Conclusion:MSKUS is an effective method to diagnose QSS. It is fast, convenient and inexpensive, and is worth popularizing in clinic.     

Acute disseminated encephalomyelitis (ADEM) onset during meningitis and sepsis

A 39-year-old man with a high fever, headache, and stiff neck, and Kernig and Brudzinski signs admitted in June 2004 had a WBC of 10,680/microL and CRP of 10.5mg/dL. Streptococcus pneumoniae was detected in blood and spinal fluid culture, but brain CT was normal. Meningitis was diagnosed and antibiotics and corticosteroids begun. After four days of treatment, his blood test and spinal fluid data had improved, but his consciousness had deteriorated. ADEM was diagnosed by the clinical course and brain MRI (T2-weighted image) that showed high-intensity lesions in the white cerebral matter, and steroid pulse treatment was begun on day 4 after admission. His consciousness disturbance rapidly disappeared and brain MRI showed that the multiple high-intensity lesions had ameliorated. The patient was released after 40 days of treatment.     

Serial CT and MRI findings in a patient with isolated angiitis of the central nervous system associated with cerebral amyloid angiopathy.

We report serial CT and MRI findings in a biopsy-proven case of cerebral amyloid angiopathy (CAA) with isolated angiitis of the central nervous system (CNS). A 69-year-old man had developed dizziness, dementia, and generalized seizure during the preceding 4 years. An initial examination by brain CT and MRI showed bilateral symmetrical periventricular lesions closely resembling those of Binswanger's disease. Subsequently, the lesions expanded slowly, involving a large area of the right cerebral hemisphere with an obvious mass effect. Since a primary brain tumor was suspected, a brain biopsy was performed, and histopathological examination revealed amyloid beta protein CAA within the meningocortical vessels associated with perivascular monocytic cuffing, indicating the presence of isolated angiitis of the CNS. Multinucleated giant cells containing intracytoplasmic beta protein amyloid around a heavily amyloid-laden cortical vessel were also observed. This is the first case report to show sequential radiographical studies of the leukoencephalopathy associated with CAA and isolated angiitis of the CNS.