This article explains the approach to managing a patient with herpes simplex virus encephalitis. Acute encephalopathy is a common and often intimidating presentation in an acute general medical setting. Application of key principles will enable the generalist to take life-saving action before obtaining any specialist input. Viral infection is the most common cause (48.2%) of encephalitis; another large group is cases of autoimmune aetiology. Early diagnosis of encephalitis is crucial to ensure that the right treatment is given on time. Guidelines on the management of viral encephalitis were published by the British Association of Neurologists and British Infection Association ( Solomon et al, 2012 ), but adherence to these standards by clinicians has been found to be suboptimal ( Han and Coebergh, 2017 ). This puts lives in danger, in the context of a treatable, serious, acute presentation. Although viral infection is the most common cause of encephalitis, an awareness of rarer forms of autoimmune encephalitis is necessary. The differential diagnosis of autoimmune encephalitis is important because the disease is potentially treatable with immunosuppressive agents. Paraneoplastic limbic encephalitis may present months or years before the detection of a tumour.
We present a case of diffuse astrocytoma mimicking the radiological features of Neuro-Behcet’s disease. A 61 year old man presented with a progressive asymmetric brainstem syndrome (left>right). Examination of cranial nerves revealed a left lateral rectus palsy, a left trigeminal sensory loss V1-V3 and left lower motor facial palsy. There were no oral or genital ulcerations, no ophthalmic symptoms or signs and no skin rashes. MR imaging showed an asymmetric mesencephalic- diencephalic hyperintensity on T2 without contrast enhancement. Cerebrospinal fluid was within normal limits including large volume cytological exam. Whole body CT and FDG-PET demonstrated no evidence of malignancy. IV methylprednisolone (1g daily) for three days was given then a 60mg prednisolone taper.He subsequently developed a right hemiparesis and was found to have on repeat MR brain a new right prefrontal cortex lesion involving the middle frontal lobe, without contrast enhancement. Biopsy showed a IDH-1 wild-type diffuse astrocytoma. He was administered chemotherapy and whole brain radiotherapy with no interval changes at 2 month follow up. Our case highlights a rare and important presentation of diffuse astrocytoma masquerading as rhombencephalitis syndrome. Timely brain biopsy allows for prompt intervention and improved performance status.christopherleahy@doctors.org.uk61
We present a case of Guillain Barre Syndrome (GBS) following resection of a brain neoplasm. A 43 y.o. man presented with diplopia and lower back pain 2 weeks after resection of left mesiotemporal and fusiform gyrus grade III IDH 1 mutant anaplastic astrocytoma. Within a week he was unable to ambulate due to marked leg weakness. Examination showed bilateral weakness of lateral rectus muscles and lower motor bilateral facial weakness. Hip flexion was 1/5 bilaterally, otherwise 0/5. Tone was flaccid. Lower limb reflexes were absent and plantar responses were mute. Imaging showed no evidence of tumour recurrence or drop metastasis. CSF analysis demonstrated raised WCC (43/ul WBCs, <1/ul RBCs), protein was markedly elevated at 4.33 g/l and glucose normal. Cytology was negative for malignancy consist- ently. He was treated 5-days of intravenous immunoglobulin (2g/kg). He walked without assistance on discharge and his recovery was sustained at 6 months follow up. Neurophysiology showed a generalised, non- length dependent, motor> sensory, demyelinating neuropathy. This case highlights the importance of considering GBS as a potential cause of progressive neurological deficit in the postoperative period where “atypical” CSF findings are confounded by the preceding neurosurgical intervention.christopherleahy@doctors.org.uk
We report on a patient with atypical parkinsonism due to coexistent Lewy body disease (LBD) and diffuse anaplastic astrocytoma. The patient presented with a mixed cerebellar and parkinsonian syndrome, incomplete levodopa response, and autonomic failure. The clinical diagnosis was multiple system atrophy (MSA). Supportive features of MSA according to the consensus diagnostic criteria included postural instability and early falls, early dysphagia, pyramidal signs, and orofacial dystonia. Multiple exclusion criteria for a diagnosis of idiopathic Parkinson's disease (iPD) were present. Neuropathological examination of the left hemisphere and the whole midbrain and brainstem revealed LBD, neocortical-type consistent with iPD, hippocampal sclerosis, and widespread neoplastic infiltration by an anaplastic astrocytoma without evidence of a space occupying lesion. There were no pathological features of MSA. The classification of atypical parkinsonism was difficult in this patient. The clinical features and disease course were confounded by the coexistent tumor, leading to atypical presentation and a diagnosis of MSA. We suggest that the initial features were due to Lewy body pathology, while progression and ataxia, pyramidal signs, and falls were accelerated by the occurrence of the astrocytoma. Our case reflects the challenges of an accurate diagnosis of atypical parkinsonism, the potential for confounding co-pathology and the need for autopsy examination to reach a definitive diagnosis.
IntroductionThe inpatient resources required to meet the needs of patients with Functional Neurological Disorders (FNDs) are uncertain, although significant contributions from neurologists, physiotherapists, psychologists and others are required. Accurate quantification of this is necessary to support care arrangement planning. We examined the inpatient activity linked to the care of patients with FNDs on a tertiary acute neurology ward to evaluate this issue.MethodsA retrospective case-note review of patients discharged from the Acute Neurology Unit at the Greater Manchester Neuroscience Centre over four months was performed. Data collected included demographics, diagnosis and length of stay.ResultsOverall 127 patients were identified. 16% had a diagnosis of FND. Of these, 60% had non-epileptic attack disorder (NEAD), 30% paralysis, 5% blindness and 5% a movement disorder. Patients with FNDs were significantly younger (mean 35 years versus 52 years, p<0.001), and more were female (85% versus 15%, p=0.005). The median length of stay for patients with FNDs was 7.5 days (IQR 5–10.5), compared to 10 days (IQR 6–21) for others (p=0.095).DiscussionPatients with FNDs, particularly NEAD, represented a significant proportion of inpatient activity. This information will support planning our FNDs service to ensure it meets the needs of this patient group.
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