Aims and method The psychiatric manifestations of Huntington's disease are myriad and difficult to control. The use of electroconvulsive therapy (ECT) is not commonly considered for this condition. We describe a patient with severe depression, psychomotor retardation, delusions and weight loss who responded to ECT with good control of her symptoms. Results Both our case and the literature appear to confirm the efficacy of ECT in the treatment of depression in Huntington's disease and suggest that other psychiatric manifestations of Huntington's are also responsive. Clinical implications ECT is an effective and safe treatment that should be considered earlier in the course of the disease in cases that show limited response to pharmacological therapy. It should also be considered as an adjunct to medical therapy that may simplify polypharmacy and allow better control in patients with debilitating psychiatric manifestations of the disease. There is limited and conflicting evidence for its efficacy in chorea.
Pre and post-herpetic SUNCT syndrome is a rare but previously reported complication of herpes zoster reactivation. We report a case of a zoster associated SUNCT syndrome developing at the same time as reactivation.A 79-year-old gentleman was referred to Neurology for refractory right sided facial pain. 7 months prior to presentation he had developed shingles in the right upper face around his forehead area, which was managed by his GP. At the same time, he developed daily attacks of sharp stabbing pain behind his eye, up to 5 a day, lasting up to 5 minutes each. This was associated with injection and lacrimation of the affected eye. The attacks can be triggered by touch, such as a shower. Neurological examination was unremarkable except for allodynia in the right V1 distribution. Plain MRI scan showed cortical atrophy only.He had a good response to lamotrigine. nchlasim@doctors.org.uk
BackgroundCerebral venous thrombosis accounts for <5% of all strokes. Cortical vein thrombosis (CVT) is even rarer and usually coexists with sinus thrombosis but can present in isolation. As a group their presentation is with headache, focal cortical signs and seizures. Radiologically, patients present with unusual patterns of ischaemia or haemorrhage (particularly subcortical) and oedema in non-arterial territories. We reviewed the potential predisposing factors to thrombosis and discuss the relevance of variation of cortical venous anatomy to CVT and clinical outcome.MethodsRetrospective case analysis of seven patients with isolated CVT. The diagnosis of which was made from a combination of the clinical picture (headache, focal cortical deficit and seizures) and the presence of direct evidence of clot or non-arterial infarction or haemorrhage on standard MRI sequences in addition to perfusion MRV and CE MRV.ResultsOf seven cases four were female, five patients presented with cerebral haemorrhage. Two patients had bilateral hemispheric involvement with three that involved the left hemisphere. Five patients presented with headache, all had focal neurological deficit and three had seizures. Five patients required heparin and six patients had good clinical outcome.ConclusionsThe presence of headache, seizures and focal cortical signs with unusual non-arterial patterns of ischaemia/haemorrhage should alert clinicians to CVT. Variation in cortical venous drainage may potentiate CVT. In the absence of prompt diagnosis and aggressive treatment with hydration and anticoagulation patients are vulnerable to poor outcome.
We report the case of a 56 year old male, presenting approximately 6 weeks after his first symptoms of Sporadic Creutzfeldt-Jakob Disease. The patient demonstrated classical magnetic resonance imaging (MRI) and electroencephalographic (EEG) changes reflecting clinical progression.Creutzfeldt-Jakob Disease was suspected and later confirmed by positive biochemical markers approximately 10 days after presentation; including positive protein 14-3-3, S-100b and positive rtQUIC assay.Serial MRI and EEG findings correlated with clinical symptoms witnessed on presentation (nystagmus, mild myoclonus and lability of mood) and disease progression through to coma before deathMRI changes were seen as initial localised cortical ribboning progressing to more widespread ribboning and areas of high signal in the basal ganglia (striatum). This correlated with the findings on his earliest EEG of periodic frontal and right side dominant sharp waves, to the last EEG showing more widespread periodic sharp waves.MRI and EEG findings are discussed as well as differentials that may appear with similar MRI findings.The presentation provides an accessible learning case for neurologist, neuro-radiologists and neurophysiologists at all levels.
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