Posts Tagged ‘Encephalitis’

Viral Encephalitis

Thursday, August 21st, 2014

Herpes simplex encephalitis

Viral encephalitis is a serious neurological disorder associated with relatively poor outcome. Herpes simplex virus (HSV) encephalitis is the most common causative agent for viral encephalitis. More than 95% of HSV encephalitis is caused by HSV type 1. HSV 1 is transmitted primarily via saliva which subsequently enters the oral mucosa and spreads to the ganglia nearby (trigeminal, superior and inferior cervical ganglia most common). Initially the virus is latent in the trigeminal ganglion. However, reactivation of the virus could occur spontaneously or could be triggered by trauma, stress, immunosuppression, or hormonal fluctuations.

Upon reactivation, there would be intracranial spread of the virus along the small meningeal braches of the trigeminal nerve, affecting the temporal lobe and orbital surface of the frontal lobe, which are the most common regions affected. The series of MRI scans here are taken from a middle-aged patient who presented with a few day history of fever and confusion (Figures 1 to 6). Lumbar puncture showed a lymphocytic predominant pleocytosis with normal CSF protein and glucose levels. Polymerase chain reaction for HSV-1 was positive. A cranial MRI subsequently demonstrated hyperintensities over bilateral temporal lobes arrow_1 (right more than left) in the T2W scan (axial cuts, Figures 1 and 2) as well as the FLAIR sequences (coronal cuts, Figures 5 and 6). There was also evidence of restricted diffusion over both temporal lobes arrow_1 (Figures 3 and 4). The patient was promptly started on intravenous acyclovir after suspicion of a central nervous system infection and continued for a total of fourteen days after confirmation by viral PCR.

Autoimmune Encephalitis

Thursday, August 21st, 2014

This cranial MRI is from a young woman who presented with low-grade fever, confusion, visual hallucinations and status epilepticus. Significant orofacial dyskinesias and autonomic dysfunction was also noted. Lumbar puncture was performed and cerebrospinal fluid (CSF) revealed mild lymphocytic predominant leukocytosis and slightly elevated protein. CSF glucose was within normal range and CSF for microbiological studies including PCR for herpes simplex encephalitis were unrevealing. Cerebral MRI revealed a hyper-intense signal involving the right medial temporal lobe and hippocampus arrow_1 in the FLAIR (Figure 1) and T2 sequences (Figure 2), compatible with limbic encephalitis. Her status epilepticus was controlled with multiple anti-epileptic agents and she was initially treated empirically as viral encephalitis with intravenous acyclovir. CSF and serum for anti-NMDA receptor antibodies were subsequently noted to be significantly positive. A diagnosis of autoimmune encephalitis due to anti-NMDA receptor antibodies was made. She was started on immunotherapy including high dose intravenous then oral steroids as well as plasmapharesis. A search for underlying malignancy was made and a right-sided ovarian teratoma was found. She was referred to the Gynaecologists for excision of the ovarian teratoma. Her steroid treatment was subsequently replaced by azathioprine, a steroid-sparing agent, and her condition gradually improved over the subsequent few weeks.

Bickerstaff’s Encephalitis

Thursday, August 21st, 2014

A middle-aged woman was admitted with drowsiness, weakness of all four limbs and visual impairment. Physical examination revealed that the patient had bilateral complete ophthalmoplegia, generalized weakness and spasticity,with brisk reflexes and bilateral upgoing plantar reflexes. A hyper-intense lesion at the level of the midbrain arrow_1 was noted on cerebral MRI (Figure 1). Cerebro-spinal fluid examination was unremarkable and microbiological workup was negative. Serum for anti-GQ1b IgG antibodies were strongly positive. A diagnosis of Bickerstaff’s encephalitis was made and the patient was treated with immunotherapy in the form of plasmapharesis, after which she made a gradual recovery.

Encephalitis

Tuesday, June 3rd, 2014

A. Clinical manifestations

The clinical manifestations of encephalitis may be similar to those of meningitis. However, altered level of consciousness is a predominant sign. It can range from mild lethargy to deep coma. Moreover, focal or generalized seizures occur in many patients with encephalitis, especially in severe disease. Virtually every possible type of focal neurological disturbances has been reported.

B. Aetiology

Encephalitis is most frequently due to viral infection. Herpes viruses (herpes simplex and varicella-zoster viruses) and enteroviruses are the commonest agents. Other viruses such as influenza virus, adenovirus, mumps virus, cytomegalovirus, Epstein-Barr virus, HIV, flaviviruses (e.g. Japanese encephalitis virus, West Nile virus) and rabies virus are potential causes.

Bacteria, fungi and parasites can also cause encephalitis – but these are far less common as a cause compared with viruses. Examples include Bartonella spp., Borrelia burgdorferi (Lyme disease), Listeria monocytogenes, Mycobacterium tuberculosis, Cryptococcus neoformans, and Toxoplasma gondii.

C. Diagnosis

Examination of the CSF usually shows pleocytosis with predominantly mononuclear cells, mildly elevated protein and normal glucose. Since the commonest aetiological agents are viral in origin, CSF should be sent for viral culture; PCR/RT-PCR for herpesviruses, enteroviruses, and other suspected viruses. In cases of suspected Japanese B encephalitis, serum and CSF should also be sent for Japanese B virus specific IgM antibodies.

Imaging studies (CT or MRI) of the brain is useful as some of the viral encephalitis have specific imaging findings. For example, in Herpes simplex virus encephalitis, temporal lobe is the most commonly affected area (Figure 1) whereas in Japanese encephalitis, the basal ganglia and thalami (especially bilaterally) are more frequently involved (Figure 2). In addition, electroencephalograms (EEG) may also reveal features suggestive of viral encephalitis, e.g. periodic lateralising epileptiform discharges.

Figure 1. Cerebral MRI of patient with HSV-2 encephalitis showing bilateral temporal lobe hyperintensity

Figure 2. Cerebral MRI of patient with Japanese B encephalitis, showing bilateral thalamic hyperintensity

D. Management

Patients with encephalitis can deteriorate rapidly and so they should be closely monitored. Complications such as increased intra-cranial pressure, seizures, and autonomic dysfunction can occur.

Viral encephalitis, the most common cause, has limited antiviral drugs with proven efficacy. Acyclovir and related compounds are the most effective antiviral agents for herpes virus infection (HSV and VZV). Supportive measures are the mainstay of treatment for most encephalitis cases.