Treatment-related fluctuation in Guillain-Barre syndrome

Guillain-Barre syndrome (GBS) is usually a monophasic illness but relapses occur. A 55-year-old female with hypertension and vitiligo presented with acute inflammatory demyelinating polyradiculoneuropathy. She improved with immunoglobulin treatment started on day 6 of illness, but relapsed on day 14 warranting repeat immunoglobulin therapy. Thereafter recovery was complete. Her relapse was due to treatment-related fluctuation (TRF). TRF is improvement in the GBS disability scale of at least one grade after completion of immunotherapy followed by worsening of the disability scale of at least one grade within the first 2 months after disease onset. Recurrent GBS and chronic inflammatory demyelinating polyradiculoneuropathy were excluded. During the peak of the illness ANA titres were transiently high. The presence of other medical conditions, predominant proximal weakness and the absence of preceding diarrhea are predictors for TRF seen in this patient. Early treatment and evidence of ongoing immune activation have contributed toward TRF

Case report Paroxysmal kinesigenic dyskinesia

Paroxysmal kinesigenic dyskinesia (PKD) is a rare disorder characterised by brief and frequent attacks of abnormal involuntary movements induced by sudden movement. This disorder has not been reported previously in Sri Lanka. We studied six patients with respect to clinical presentation, aetiology, family history and response to treatment, and describe the Sri Lankan patterns of this illness. All the patients were males and the age at onset was from 11 to 22 years. The involuntary movements in all were dystonic and affected one or both sides, involving the face in the majority. All had difficulty in speaking during the attacks. One patient had an occasional attack during exercise. In all, the illness was sporadic, none had a family history of a similar illness and in none was it due to a secondary cause. The attacks usually lasted 10–60 seconds, and occurred up to 20 times a day. All patients responded well to anticonvulsants. PKD in Sri Lanka has a pattern similar to that described worldwide

Idiopathic hypertrophic pachymeningitis presenting with a superficial soft tissue mass

Idiopathic hypertrophic pachymeningitis (IHP) is a chronic progressive diffuse inflammatory fibrosis of the duramater, leading to its diffuse enlargement. The following describes a case of IHP presenting with a superficial soft tissue mass. A 40-year-old female came to hospital with a subcutaneous lump over the left face and frontal headache for 6 months. An excision biopsy revealed chronic inflammation. Magnetic resonance imaging (MRI) of the brain showed left mastoiditis and early dural inflammation of the left temporal region. A few months later, she developed diplopia, complex partial seizures, and retrobulbar neuritis of the left optic nerve. Repeat MRI brain demonstrated meningeal thickening on both sides of the tentorium cerebelli extending to the left tempero-parietal meninges. The meningeal biopsy revealed markedly thickened fibro-connective dural tissue with infiltration of chronic inflammatory cells. There was no evidence of bacterial, fungal, tuberculous or neoplastic infiltration. IHP was diagnosed and steroid therapy initiated. Within weeks, she showed marked clinical improvement. IHP is a diagnosis of exclusion. The absence of underlying infective, neoplastic, or systemic autoimmune disease favors IHP. The above patient had headache, neuro-ophthalmic signs, seizures, which are features of IHP. However, superficial soft tissue involvement is rare

Viral aetiologies of acute encephalitis in a hospital-based South Asian population

Abstract Background The aetiological spectrum of acute encephalitis shows inter- and intra-geographical variations. We aimed to identify the viruses that cause infectious encephalitis in Sri Lanka, which represents a South Asian population. Methods A cross-sectional study was conducted among 99 patients with encephalitis/meningoencephalitis admitted to two tertiary-care hospitals in Colombo. Cerebrospinal fluid and serum were tested for conventional and emerging encephalitogenic viruses. Specific nucleic acid amplification and antibody assays were used to identify viruses. Plaque reduction neutralization test was done to confirm the diagnosis of West Nile virus (WNV). Results Patients’ age ranged from 1 month to 73 years (mean = 24.91; SD = 21.33) with a male:female ratio of 1.75:1. A viral aetiology was identified in only 27.3%. These included dengue virus (40.7%), Japanese encephalitis virus (25.9%), varicella zoster virus, WNV and probable Epstein Barr virus (11.1% each). None were positive for herpes simplex viruses or cytomegalovirus. Screening for bacterial aetiologies was negative for all patients. There were no distinguishable clinical or laboratory findings between the different viral aetiologies. The case fatality rate was 7%, which was higher among patients with an identified viral aetiology. Conclusions A viral aetiology was identified in only about a quarter of patients with encephalitis. Dengue virus accounted for the majority