Annals of Indian Academy of Neurology

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Annals of Indian Academy of Neurology

Annals of Indian Academy of Neurology

@AnnIndAcadNeur

Official handle of the Annals of Indian Academy of Neurology (AIAN). AIAN is the flagship journal of Indian Academy of Neurology

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Annals of Indian Academy of Neurology
The presence of pontine stripes, superior vermian atrophy or thin corpus callosum in a patient with spastic ataxia should raise suspicion for ARSACS. It can be associated with a demyelinating neuropathy. Link: 10.4103/aian.AIAN_16_20 @neuro_ian #ARSACS
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Biotin‑thiamine‑responsive basal ganglia disease is a rare, AR, treatable disorder due to mutations in SLC19A3 gene. Presents as an early‑childhood encephalopathy +/- lactic acidosis +/- infantile spasms. Rx is life long: high dose biotin and thiamine. @neuro_ian @Dr_Suvasini
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Maramattom et al highlight DADA2 mutations as a rare cause of stroke in young. Can cause both ischemic & hemorrhagic stroke and should be strongly suspected in the presence of livedo racemosa. TNF alpha inhibitors are the drug of choice. Link: 10.4103/aian.AIAN_716_20 @neuro_ian
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Apkari et al reported that neuroimaging in FIRES evolves over time. Bilateral basal ganglia and hippocampal involvement occurs initially. The "claustrum sign" and cortical involvement is seen a few days later @neuro_ian #FIRES Link: 10.4103/aian.aian_823_23
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Koshy et al published the largest series (n=33) of spontaneous intracranial hypotension from India. Orthostatic headache and pachymeningeal enhancement were the most common findings. Extradural blood patch application led to rapid relief @neuro_ian Link: 10.4103/aian.aian_1313_25
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Can we give a probable diagnosis of muscular dystrophies in the absence of genetic testing? @vishnuvy and Garg et al report that different dystrophies have distinguished MRI phenotypes (MRI may serve as a biomarker) making this possible @neuro_ian Link: 10.4103/aian.aian_1092_25
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Adrenoleukodystrophy is associated with posterior predominant involvement of white matter. Saini et al reported that the occurrence of peritrigonal and periventricular calcifications (doing a CT with MRI) may be a radiological clue to its diagnosis. Link: 10.4103/aian.AIAN_974_20
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Manganese neurotoxicity is predominantly described in children and adolescents. Involves mutations in SLC30A10 & SLC39A14 genes. Exhibit dystonia & parkinsonism with relatively preserved cognition. Sharma et al published what neurologists should know. 10.4103/aian.AIAN_789_20
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Tuberous sclerosis (TS) is associated with myriad clinical features including retinal astrocytoma, cortical tubers, adenoma sebaceum and shagreen patches. Rana et al described one such case with all these classical findings. Link: 10.4103/aian.AIAN_590_20
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Takayasu arteritis is a large vessel vasculitis predominantly affecting young women. Subclavian steal phenomenon can occur by the predominant affliction of the left subclavian artery (before left VA origin). George et al has described one such case. Link: 10.4103/aian.AIAN_25_22
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Saraf et al report that the interpeduncular angle (IPA) may help in differentiating between IPD and Atypical Parkinsonian Syndromes (APS), especially in the middle aged population. APS were found to have higher IPA (>70*) than IPD. @movedisorder Link: 10.4103/aian.aian_357_25
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Lichenoid drug reactions (LDRs) are classified as type IV delayed hypersensitivity. LDRs are classically associated with penicillamine, beta blockers, anti-malarials and thiazide diuretics. Agharbi et al describe a rare case of LDR with Teriflunamide in an MS patient
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Hirayama disease (HD) characteristically presents with a progressive LMN syndrome. Nalini et al report that it can rarely present with a predominant UMN syndrome. HD can also mimic ALS; should be ruled out in young males with pure motor quadriparesis. 10.4103/aian.aian_922_22
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Heerfordt-Waldenstrom syndrome (HWS) is a tetrad of fever, parotid enlargement, facial palsy and anterior uveitis. HWS is rare but considered pathognomonic of sarcoidosis. @drayushagarwal has described one such case. Link: 10.4103/aian.aian_1014_25
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L-2 hydroxy glutaric aciduria is characterized by the involvement of subcortical WM and cerebellar dentate nucleus +/- basal ganglia and cerebral/cerebellar atrophy. Thalamus and brainstem are usually spared. Saini et al describe one such case. Link: 10.4103/aian.aian_1070_25
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Hot off the press! The "trident sign" is not sine qua non with neurosarcoidosis. @ranjot777 et al describe this sign in a patient with aquaporin 4 antibody positive NMOSD. #NMOSD Link: 10.4103/aian.aian_1130_25
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