Venugopalan Y. Vishnu

A test for Creutzfeldt-Jakob disease using nasal brushings.

n engl j med 371;19 nejm.org november 6, 2014 1842 with an average decrease of 5 points in full-scale IQ in children with sickle cell anemia,1 we designed the primary analysis to include the recurrence of overt and silent infarcts. Adhering to guidelines of reporting subgroup analyses in clinical trials,2 we did not analyze the data according to the suggested strategy of comparing the incidence rate of strokes or silent cerebral infarcts separately. The 2002 NIH guidelines state that the optimal frequency of rescreening with a transcranial Doppler measurement is not established.3 Our use of rescreening at 12 to 18 months provided a practical transcranial Doppler screening window for a complex clinical trial. Our results definitively show the benefit of blood-transfusion therapy in preventing the recurrence of infarcts, particularly since the intention-to-treat analysis and the crossover rate of 15% from the transfusion to the observation group both bias the results toward the null hypothesis. Had we decreased the rescreening interval to 12 months, we do not believe our primary results would have changed.

Is Neurobrucellosis the Pandora's Box of Modern Medicine?

TO THE EDITOR—The recent article by Guven et al [1], titled “Neurobrucellosis: Clinical and Diagnostic Features,” sheds light on many important yet vexing issues in the clinical presentation, diagnosis, andmanagementofarelativelycommon zoonotic infection. The relevance of discussing this issue is understated by the lack of consensus in the diagnosis and management of neurobrucellosis, in combination with unchanged diagnostic and therapeutic approaches for brucellosis being practiced for decades.Wewould like to express our views regarding certain aspects in the diagnosis and management of neurobrucellosis. The widely followed diagnostic criteria overemphasizes the role of abnormal cerebrospinal fluid (CSF) (positive Brucella agglutination titer in any titer, isolation of Brucella from CSF, and abnormal CSF parameters) in the diagnosis of neurobrucellosis [2]. However, in resourcepoor settings such as ours, facilities for determination of CSF Brucella agglutination titers are sparse. Due to the long

A motor neuron disease.

IMPORTANCE Allgrove syndrome is a rare autosomal recessive disorder characterised by achalasia, alacrima, adrenal insufficiency, autonomic dysfunction and amyotrophy. The syndrome has been described in childhood and adult presentation, as in our case, is very rare. There is a considerable delay in diagnosis due to lack of awareness about the syndrome. OBSERVATIONS We report a single case of a 36 year old man who was initially diagnosed and treated for achalasia cardia in our institute 14 years before. After 8 years he presented again with weakness and wasting predominantly distally. He had tongue fasciculations, brisk reflexes and extensor plantar. After supportive electrophysiological studies he was diagnosed as Amyotrophic lateral sclerosis. After 5 years he presented with generalised fatigue without any significant worsening of his neurological status. On reevaluation he had alacrimia, autonomic dysfunction and mild ACTH resistance. CONCLUSIONS AND RELEVANCE Allgrove syndrome may be an underdiagnosed cause of multisystem neurological disease due to the heterogeneous clinical presentation as well as for ignorance of clinician about the syndrome. Based on our case, we also believe that there does exist a subgroup of patients who follow a less severe and chronic course. Recognition of syndrome allows for treatment of autonomic dysfunction, adrenal insufficiency and dysphagia.

Can tauopathy shake the amyloid cascade hypothesis

that reported neuronal injury in the absence of substantial amyloid burden. After two decades of chasing evi-dence for the amyloid hypothesis of AD, and after the recent disappointments in immunotherapy trials, it is imperative that alternative hypotheses are considered and actively pursued. New models are emerging that present opportunities for therapeutic research. The amyloid hypothesis was again questioned in the article by Chetelat.

Parkinson syndrome: A precise localization for abducens palsy

BACKGROUND Unilateral isolated abducens palsy can occur due to any lesion from pons to orbit. The precise localization is made through the associated neurological signs. Parkinson syndrome is a symptom complex of unilateral abducens palsy with ipsilateral postganglionic Horner syndrome localizing the lesion to posterior cavernous sinus. RESULTS We describe here a 55 year old lady who presented with headache and diplopia for 3 months. On examination she had right lateral rectus palsy and postganglionic Horner syndrome. No other neurological deficit was present. MRI brain and MRA of intracranial vessels showed aneurysm of the right cavernous internal carotid artery which was confirmed on cerebral angiography. Endovascular coiling was advised but refused by the patient and she was treated symptomatically CONCLUSIONS Parkinson syndrome gives precise localization to unilateral abducens palsy and hence is a valuable clinical pearl.

Role of Plasma Clusterin in Alzheimer’s Disease—A Pilot Study in a Tertiary Hospital in Northern India

Objective To evaluate the role of plasma clusterin in Alzheimer’s disease (AD). Background Plasma clusterin is a promising biomarker as various studies have shown it to be associated with AD. But other studies have shown that plasma clusterin levels were not related to Alzheimer’s disease or presymptomatic AD. Hence the diagnostic value of plasma clusterin is still not conclusive. Methods Neuropsychological assessment, MRI brain, FDG-PET brain and CSF biomarkers of AD were used for establishing the diagnosis of MCI, AD or Vascular dementia. The CSF control group included patients who were having knee or hip surgery and plasma control group included the spouses of patients. Results Forty-six patients who gave consent for CSF examination and FDG PET brain were included in the study along with 19 control samples. Alzheimer’s group had 34 patients and Vascular group had 12 patients. Both had a significantly lower value of clusterin than the control samples (p<0.01). The median plasma clusterin level was 84.38 μg/ml in control group, 57.98μg/ml in Alzheimer’s group and 49.93μg/ml in the vascular group. Alzheimer and Vascular group did not differ in plasma clusterin levels. Moreover there was no correlation of plasma clusterin with AD severity. The sensitivity and specificity of plasma clusterin was low for any significance for clinical use. Conclusion Our pilot study shows that plasma clusterin is lower in Alzheimer’s disease with respect to control population. Plasma clusterin levels and severity of Alzheimer’s disease had no significant correlation. There was no difference in plasma clusterin between Alzheimer’s disease and Vascular Dementia. The sensitivity and specificity of plasma clusterin is low for any use in clinical practice. More studies are required to ascertain the utility of plasma clusterin as a biomarker in Alzheimer’s disease.

Fatal disseminated neurobrucellosis.

A 59-year-old cattle breeder hailing from rural north western India presented with insidious onset and gradually progressive hoarseness of voice and painless dysphagia for 5 months followed by ascending paraparesis and hearing loss for 2 months. He had significant loss of weight with anorexia of 5 months duration. Physical examination revealed pallor, bilateral 7th, 8th, 9th, 10th and left 12th cranial nerve palsies with sensorimotor deficits in radicular distribution (L3-S4) and neck stiffness. Hemogram showed elevated erythrocyte sedimentation rate of 42 mm in …

Disseminated tuberculosis presenting as rapidly progressive dementia.

A 35-year-old right-handed man presented with complaints of holocranial nonthrobbing headache with apathy, executive dysfunction and reduced social interaction for the past 1 month. No relevant systemic symptoms were present. General physical and neurological examination was unremarkable. Hematological parameters were normal except for raised erythrocyte sedimentation rate (52 mm in first hour). Gad-enhanced magnetic resonance imaging (MRI) brain showed acute left thalamic infarct …

Sporadic hypokalemic paralysis caused by osmotic diuresis in diabetes mellitus

A wide variety of neurological manifestations are known in patients with diabetes mellitus. We describe a 40-year-old man who presented with hypokalemic paralysis. On evaluation, we found that the cause of the hypokalemia was osmotic diuresis induced by marked hyperglycemia due to undiagnosed diabetes mellitus. The patient had an uneventful recovery with potassium replacement, followed by glycemic control with insulin. Barring a few instances of symptomatic hypokalemia in the setting of diabetic emergencies, to our knowledge uncomplicated hyperglycemia has not been reported to result in hypokalemic paralysis.

Implications of presymptomatic change in thalamus and caudate in Alzheimer’s disease

ARTICLE Sir, I read with interest the article by Ryan et al. (2013) on MRI evidence for presymptomatic change in the thalamus and caudate in familial Alzheimer’s disease. Atrophy is thought to be a biomarker for neuronal loss, which comes relatively far down in the amyloid cascade hypothesis as a downstream element. Ryan et al. (2013) showed that atrophy in the caudate and thalamus can arise years before the hippocampal atrophy. Is this a marker of amyloid-induced neuronal injury …