Medullary Hot-Cross Bun Sign in Multiple System Atrophy-Cerebellar: A new place for holy cross!
Multiple-system wasting ( MSA ) is a progressive neurodegenerative upset. Consensus standards clinically classify MSA patients into parkinsonian ( MSA-P ) and cerebellar ( MSA-C ) subtypes. It causes disfunction of multiple systems including autonomic, extrapyramidal, pyramidic and cerebellar system. Pontine hot-cross roll ( HCB ) mark in MSA is a well-documented and extremely specific entity due to selective loss of medullated pontocerebellar fibres and nerve cells in pontine rhaphe. To the best of our cognition, medullary hot-cross roll ( HCB ) mark has ne’er been described in literature. Herein, we report a instance of MSA-C with medullary HCB mark. Detection of medullary HCB mark might be a foster marker in the diagnosing of MSA-C, peculiarly in the absence of classical pontine HCB mark, nevertheless farther research will assist to formalize this mark.
Cardinal Wordss:Hot-cross roll mark, Multiple system wasting, Paralysis agitans
Multiple system wasting ( MSA ) is a sporadic, adult-onset, progressive neurodegenerative upset. It causes disfunction of multiple systems including autonomic, extrapyramidal, pyramidic and cerebellar system. Consensus standards clinically classify MSA patients into parkinsonian ( MSA-P ) and cerebellar ( MSA-C ) subtypes. Sometimes, distinction from idiopathic Parkinson’s disease ( PD ) can be hard due to overlap characteristics. Therefore, accessory diagnostic trials are utile in such state of affairss. Pontine hot-cross roll ( HCB ) visual aspect on magnetic resonance imagination is a well-documented and extremely specific mark in MSA. It is because of selective loss of medullated pontocerebellar fibres and nerve cells in pontine rhaphe. However, hot-cross roll ( HCB ) mark is non seen in all MSA patients. HCB mark and hyperintensities of in-between cerebellar peduncle ( MCP ) on T2-Weighted MRI could give sensitiveness of 85 % and specificity of 100 % to distinguish between MSA-C and PD [ 2, 3 ] . Here, we report a instance of MSA-C with medullary HCB mark.
A 54-year-old male presented with bit by bit progressive trouble in walking, inclination to rock on either side, awkwardness in the day-to-day activities of life, anhydrosis, postural blackouts, urinary frequence and erectile disfunction for three old ages. He had no hallucinations, fluctuating symptoms, forgetfulness, falls, injury or illicit drug maltreatment. There was no history of diabetes mellitus, high blood pressure or such type of unwellness in household. On scrutiny, patient had important orthostatic hypotension. Mini mental position scrutiny ( MMSE ) consequences were normal. Saccade and chase motions were normal without optic regard limitation. He had bilateral symmetrical bradykinesia, gear rigidness and generalized hyperreflexia with extensor plantar responses. Pancerebellar marks including scanning address, purpose shudders, finger-to-nose and heel-shin incoordination, appendicular and truncal ataxy were present. R-R interval on EKG showed autonomic disfunction.
Hemogram, serum biochemistry including vitamin B12 and thyroid profile were normal. Serum ELISA trial for human immunodeficiency virus ( HIV ) was negative. MRI encephalon T2 weighted and fluid attenuated inversion recovery ( FLAIR ) images showed mild wasting of myelin, Ponss, cerebellum, in-between cerebellar peduncles and the intellectual cerebral mantle [ Figure 1 ] . Surprisingly, a alone cruciate shaped ( hot-cross roll ) hyperintense signal on FLAIR image was noted in the myelin [ Figure 2 and 3 ] . Treatment was initiated with levodopa + carbidopa ( 125 milligram thrice a twenty-four hours ) ; nevertheless, there was no benefit of this intervention. Other diagnostic medical intervention and physical therapy were besides given. He is still ambulatory with support at annual follow-up.
Multiple system wasting ( MSA ) is a neurodegenerative disease with glial cytoplasmatic inclusion organic structures incorporating alpha synuclein protein. Clinical characteristics can be a combination of cerebellar, independent and parkinsonian symptoms. It has two subtypes: cerebellar ( MSA-C ) and parkinsonian ( MSA-P ) . Our patient fulfilled the clinical diagnostic standards of likely multiple system wasting ( MSA-C ) [ 1 ] . Other differential diagnosing like, progressive supranuclear paralysis ( PSP ) , corticobasal ganglionic devolution ( CGD ) and Lewy organic structure dementedness ( LBD ) were ruled out clinically and by imaging findings as there was no gaze palsy, apraxia and asymmetric cortical wasting, and absence of dementedness and normal mini mental scrutiny ( MMSE ) , severally.
Pontine hot-cross roll ( HCB ) mark in MSA is a well-documented and extremely specific entity due to selective loss of medullated pontocerebellar fibres and nerve cells in pontine rhaphe [ 2 ] . Pontine HCB mark is neither ever seen nor a pathognomonic in MSA patients. It is besides seen in spinocerebellar ataxy, cerebrotendinous xanthoma multiplex [ 4 ] , paralysis agitans secondary to vasculitis [ 4 ] , human immunodeficiency virus ( HIV ) [ 6 ] and variant Creutzfeldt-Jakob disease ( vCJD ) [ 7 ] .
To the best of our cognition, medullary HCB mark has ne’er been described in literature. The possible mechanism may be related to selective wallerian devolution of medullated traversing fibres and nerve cells in inferior cerebellar peduncles including spinocerebellar and olivocerebellar piece of lands. Detection of medullary HCB mark might be a foster marker in the diagnosing of MSA-C, peculiarly in the absence of classical pontine HCB mark, nevertheless farther research will assist to formalize this mark.
Figure 1. MRI encephalon FLAIR image demoing mild wasting of Ponss, cerebellum, in-between cerebellar peduncles and intellectual cerebral mantle. There was no cruciform hyperintensity in the Ponss.
Figure 2. MRI encephalon FLAIR image demoing a cruciform hyperintense signal ( hot-cross roll ) in the myelin.
Figure 3. Magnified position of MRI encephalon FLAIR image demoing hot-cross roll in myelin.
1. Gilman S, Wenning GK, Low PA, Brooks DJ, Mathias CJ, Trojanowski JQ, et Al. Second consensus statement on the diagnosing of multiple system wasting. Neurology. 2008 ; 71 ( 9 ) :670-676.
2. Schrag A, Kingsley D, Phatouros C, Mathias CJ, Lees AJ, Daniel SE, et Al. Clinical utility of magnetic resonance imagination in multiple system wasting. J Neurol Neurosurg Psychiatry. 1998 ; 65:65-71.
3. Pastakia B, Polinsky R, Di Chiro G, Simmons JT, Brown R, Wener L. Multiple system wasting ( Shy-Drager syndrome ) : MR imagination. Radiology. 1986 ; 159 ( 2 ) :499-502.
4. Jain RS, Sannegowda RB, Agrawal A, Hemrajani D, Jain R, Mathur T. ‘Hot cross roll ‘ mark in a instance of cerebrotendinous xanthoma multiplex: a rare neuroimaging observation. BMJ Case Rep. 2013 Feb 14 ; 2013.
5. Muqit MM, Mort D, Miskiel KA, Shakir RA. “ Hot cross roll ” mark in a patient with paralysis agitans secondary to assume vasculitis. J Neurol Neurosurg Psychiatry. 2001 ; 71 ( 4 ) :565-566.
6. Jain RS, Nagpal K, Tejwani S. ‘Hot-cross roll ‘ and ‘inverse trident mark ‘ in progressive multifocal leukoencephalopathy with HIV seropositivity. Neurol India 2014 ; 62:341-342.
7. Soares-fernandes JP, Ribeiro M, Machado A. “ Hot cross roll ” mark in variant Creutzfeldt-Jakob disease. AJNR Am J Neuroradiol. 2009 ; 30 ( 3 ) : E37.