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{{WBRQuestion
{{WBRQuestion
|QuestionAuthor={{Rim}}
|QuestionAuthor= {{Rim}} (Reviewed by  {{YD}})
|ExamType=USMLE Step 1
|ExamType=USMLE Step 1
|MainCategory=Microbiology, Pathophysiology
|MainCategory=Pathophysiology
|SubCategory=Neurology, Infectious Disease
|SubCategory=Neurology
|MainCategory=Microbiology, Pathophysiology
|MainCategory=Pathophysiology
|SubCategory=Neurology, Infectious Disease
|SubCategory=Neurology
|MainCategory=Microbiology, Pathophysiology
|MainCategory=Pathophysiology
|SubCategory=Neurology, Infectious Disease
|SubCategory=Neurology
|MainCategory=Microbiology, Pathophysiology
|MainCategory=Pathophysiology
|MainCategory=Microbiology, Pathophysiology
|MainCategory=Pathophysiology
|SubCategory=Neurology, Infectious Disease
|MainCategory=Pathophysiology
|MainCategory=Microbiology, Pathophysiology
|SubCategory=Neurology
|SubCategory=Neurology, Infectious Disease
|MainCategory=Pathophysiology
|MainCategory=Microbiology, Pathophysiology
|SubCategory=Neurology
|SubCategory=Neurology, Infectious Disease
|MainCategory=Pathophysiology
|MainCategory=Microbiology, Pathophysiology
|SubCategory=Neurology
|SubCategory=Neurology, Infectious Disease
|MainCategory=Pathophysiology
|MainCategory=Microbiology, Pathophysiology
|SubCategory=Neurology
|MainCategory=Microbiology, Pathophysiology
|MainCategory=Pathophysiology
|SubCategory=Neurology, Infectious Disease
|MainCategory=Pathophysiology
|Prompt=A 55 year old woman with poor medical follow-up is referred to the neurology clinic after she was found an abnormal neurological exam in a local healthcare fair. Upon questioning the patient reports several months of lower extremity numbness and imbalance. She also reports shooting pains in her abdomen and back which she thought were due to indigestion. On physical exam you notice bilateral miotic pupils, non-reactive to light, but reactive to accomodation. You also observe diminished reflexes in lower extremities, and a wide-based and slapping gait. As part of your work-up you order a lumbar puncture that shows WBCs=53/mcL and a positive RPR. Which of the following images depict the lesion(s) that can be seen in this patient?  
|SubCategory=Neurology
|Explanation=Tabes dorsalis is a neurodegenerative disease seen in tertiary neurosyphilis that involves the posterior columns and roots of the spinal cord. The presentation is usually that of loss of proprioceptive sensations with progressive ataxia that can lead to paralysis if untreated. Lightning-like shooting pains are also common. Physical exam usually reveals a wide based gait, diminished reflexes and loss of vibration and position senses in the lower extremities. Argyll Robertson pupils that are baseline miotic pupils non-reactive to light but reactive to accommodation can also be seen. CSF usually shows leukocytosis >20 cells/mcL and positive VDRL or RPR. Tabes dorsalis is a rare disease since the antibiotic era although some cases can still be seen since the onset of this syndrome is 20-30 years after the initial infection. Treatment with penicillin stops the progression but cannot reverse the damage done.
|Prompt=A 55-year-old woman with poor medical follow-up presents with ulcers on the soles of her feet. Upon questioning, the patient reports lower extremity numbness and imbalance for the past 3 months. She also complains of occasional urinary incontinence and shooting pains in her abdomen and back, which she sometimes attributed to indigestion. On physical examination, the physician notices bilateral miotic pupils that are non-reactive to light but are reactive to accomodation. He also notes the presence of foot ulcers, diminished reflexes in the lower extremities, and a wide-based, slapping gait. Which of the following images depict the spinal cord lesions that are most likely responsible for the patient's condition (lesion in red)?
 
|Explanation=[[Tabes dorsalis]] is a parenchymatous neurosyphilis disorder that is characterized by progressive degeneration of the posterior columns, roots, and ganglia of the spinal cord, which usually carry the proprioception, pressure, vibration, and touch sensory pathways. Manifestations of tabes dorsalis classically include shooting pains, ataxia, areflexia, Argyll-Roberston pupils (also called prostitute pupils because they accomodate but do not react), and positive Romberg sign. Other non-specific features may include bowel and bladder dysfunction such as urinary incontinence, gastric pains, impotence, visual loss, Charcot's arthopathy, wide-based slapping gate, and foot ulcerations. Unlike other forms of CNS involvement in syphilis, (eg syphilis meningitis), the hallmark of tabes dorsalis is the onset of symptoms several years following the initial exposure to syphilis. The delay period from initial exposure to the development of tabes dorsalis may take up to 5-30 years if syphilis was left untreated. In tabes dorsalis, positive RPR or VDRL in the serum and in the CSF are suggestive of previous or current neurosyphilis. In contrast, isolation of ''T. pallidum'', the organism responsible for syphilis, from CSF in tabes dorsalis is unusual, suggesting a rather immunological disorder that affects the dorsal columns of the spinal cord in tabes dorsalis due to untreated syphilis rather than an infectious process. Treatment with [[penicillin]] stops the progression of tabes dorsalis but cannot reverse the injury that has already been made.
Educational Objective:
|AnswerA=[[Image:WBR0570C.gif|300px]]
Tabes dorsalis is disease seen in tertiary syphilis that leads to destruction of the dorsal columns and roots.
|AnswerAExp=This image depicts the involvement of the dorsal columns of the [[spinal cord]] that carry the ascending [[proprioception]], pressure, touch, and vibration sensory pathways. Involvement of the posterior columns, roots, and ganglia of the spinal cord is characteristic of tabes dorsalis caused by neurosyphilis.
 
|AnswerB=[[Image:WBR0570E.gif|300px]]
References:
|AnswerBExp=This image typically depicts spinal cord lesions consistent with ALS. ALS may involve both the descending corticospinal tracts (upper motor neurons) and the anterior horns (lower motor neurons). The involvement of both the upper and lower motor neuron pathways is the hallmark of ALS.
Lanska DJ. The suspension apparatus for tabes dorsalis. Neurology. Apr 12 1999;52(6):1295.
|AnswerC=[[Image:WBR0570D.gif|300px]]
Chahine LM, Khoriaty RN, Tomford WJ, Hussain MS. The changing face of neurosyphilis. Int J Stroke. Apr 2011;6(2):136-43. [Medline]
|AnswerCExp=This image depicts isolated anterior horn injury suggestive of [[poliomyelitis]] or [[Werdnig-Hoffman syndrome]] (floppy baby syndrome).
|AnswerA=[[Image:Dorsal-Columns.gif|300px]]
|AnswerD=[[Image:WBR0570B.gif|300px]]
|AnswerAExp=This image depicts the involvement of the dorsal columns of the spinal cord that house the ascending proprioception and vibration sense pathways. The dorsal columns are affected in tabes dorsalis but are also be involved in other syndromes including subacute combined degeneration seen with Vitamin B12 and Vitamin E deficiency.
|AnswerDExp=This image depicts multiple, non-patterned white matter lesions characteristic of [[multiple sclerosis]].
|AnswerB=[[Image:ALS.gif|300px]]
|AnswerE=[[Image:WBR0570A.gif|300px]]
|AnswerBExp=This image typically depicts spinal cord lesions of ALS that involve both descending corticospinal tracts, and the anterior horns. The involvement of both these structures is what leads to the combination of UMN and LMN symptoms seen in ALS.
|AnswerEExp=This image is depicts an expanding central canal compressing adjacent tracts typically seen in [[syringomyelia]]. The anterior white commissure is the closest structure which leads to compression of the decussating spinothalamic tracts first.
|AnswerC=[[Image:Anterior-horn.gif|300px]]
|EducationalObjectives=[[Tabes dorsalis]] is a parenchymatous neurosyphilis disorder that is characterized by progressive degeneration of the posterior columns, roots, and ganglia of the spinal cord.
|AnswerCExp=This image depicts isolated anterior horn injury usually seen in patients with poliomyelitis or Werdnig-Hoffman Syndrome (Floppy Baby Syndrome). Both of these diseases only cause LMN signs due to the lack of involvement of descending motor tracts.
|References=Lanska DJ. The suspension apparatus for tabes dorsalis. Neurology. 1999;52(6):1295.<br>
|AnswerD=[[Image:MS.gif|300px]]
Chahine LM, Khoriaty RN, Tomford WJ, Hussain MS. The changing face of neurosyphilis. Int J Stroke. 2011;6(2):136-43.<br>
|AnswerDExp=This image depicts multiple, non-patterned white matter lesions usually seen in patients with multiple sclerosis.
Karibasappa NA, Ragunatha S, AnnuJayan MP. Tabes dorsalis - a case report. Indian J Sex Transm Dis. 2006;27(1):24.<br>
|AnswerE=[[Image:Syringomyelia spinal cord.gif|300px]]
First Aid 2014 page 141, 467
|AnswerEExp=This image is depicts an expanding central canal compressing adjacent tracts typically seen in syringomyelia. The anterior white commissure is the closest structure which leads to compression of the decussating spinothalamic tracts first.
|RightAnswer=A
|RightAnswer=A
|WBRKeyword=Tabes dorsalis, syphilis, dorsal columns
|WBRKeyword=Tabes dorsalis, Syphilis, Dorsal columns, Posterior columns, Proprioception, Pressure, Vibration, Touch, Romberg, Argyll-Robertson pupil, Argyll, Robertson, Pupil, Pupils
|Approved=No
|Approved=No
}}
}}

Latest revision as of 01:01, 28 October 2020

 
Author [[PageAuthor::Rim Halaby, M.D. [1] (Reviewed by Yazan Daaboul, M.D.)]]
Exam Type ExamType::USMLE Step 1
Main Category MainCategory::Pathophysiology
Sub Category SubCategory::Neurology
Prompt [[Prompt::A 55-year-old woman with poor medical follow-up presents with ulcers on the soles of her feet. Upon questioning, the patient reports lower extremity numbness and imbalance for the past 3 months. She also complains of occasional urinary incontinence and shooting pains in her abdomen and back, which she sometimes attributed to indigestion. On physical examination, the physician notices bilateral miotic pupils that are non-reactive to light but are reactive to accomodation. He also notes the presence of foot ulcers, diminished reflexes in the lower extremities, and a wide-based, slapping gait. Which of the following images depict the spinal cord lesions that are most likely responsible for the patient's condition (lesion in red)?]]
Answer A [[AnswerA::]]
Answer A Explanation [[AnswerAExp::This image depicts the involvement of the dorsal columns of the spinal cord that carry the ascending proprioception, pressure, touch, and vibration sensory pathways. Involvement of the posterior columns, roots, and ganglia of the spinal cord is characteristic of tabes dorsalis caused by neurosyphilis.]]
Answer B [[AnswerB::]]
Answer B Explanation [[AnswerBExp::This image typically depicts spinal cord lesions consistent with ALS. ALS may involve both the descending corticospinal tracts (upper motor neurons) and the anterior horns (lower motor neurons). The involvement of both the upper and lower motor neuron pathways is the hallmark of ALS.]]
Answer C [[AnswerC::]]
Answer C Explanation [[AnswerCExp::This image depicts isolated anterior horn injury suggestive of poliomyelitis or Werdnig-Hoffman syndrome (floppy baby syndrome).]]
Answer D [[AnswerD::]]
Answer D Explanation [[AnswerDExp::This image depicts multiple, non-patterned white matter lesions characteristic of multiple sclerosis.]]
Answer E [[AnswerE::]]
Answer E Explanation [[AnswerEExp::This image is depicts an expanding central canal compressing adjacent tracts typically seen in syringomyelia. The anterior white commissure is the closest structure which leads to compression of the decussating spinothalamic tracts first.]]
Right Answer RightAnswer::A
Explanation [[Explanation::Tabes dorsalis is a parenchymatous neurosyphilis disorder that is characterized by progressive degeneration of the posterior columns, roots, and ganglia of the spinal cord, which usually carry the proprioception, pressure, vibration, and touch sensory pathways. Manifestations of tabes dorsalis classically include shooting pains, ataxia, areflexia, Argyll-Roberston pupils (also called prostitute pupils because they accomodate but do not react), and positive Romberg sign. Other non-specific features may include bowel and bladder dysfunction such as urinary incontinence, gastric pains, impotence, visual loss, Charcot's arthopathy, wide-based slapping gate, and foot ulcerations. Unlike other forms of CNS involvement in syphilis, (eg syphilis meningitis), the hallmark of tabes dorsalis is the onset of symptoms several years following the initial exposure to syphilis. The delay period from initial exposure to the development of tabes dorsalis may take up to 5-30 years if syphilis was left untreated. In tabes dorsalis, positive RPR or VDRL in the serum and in the CSF are suggestive of previous or current neurosyphilis. In contrast, isolation of T. pallidum, the organism responsible for syphilis, from CSF in tabes dorsalis is unusual, suggesting a rather immunological disorder that affects the dorsal columns of the spinal cord in tabes dorsalis due to untreated syphilis rather than an infectious process. Treatment with penicillin stops the progression of tabes dorsalis but cannot reverse the injury that has already been made.

Educational Objective: Tabes dorsalis is a parenchymatous neurosyphilis disorder that is characterized by progressive degeneration of the posterior columns, roots, and ganglia of the spinal cord.
References: Lanska DJ. The suspension apparatus for tabes dorsalis. Neurology. 1999;52(6):1295.
Chahine LM, Khoriaty RN, Tomford WJ, Hussain MS. The changing face of neurosyphilis. Int J Stroke. 2011;6(2):136-43.
Karibasappa NA, Ragunatha S, AnnuJayan MP. Tabes dorsalis - a case report. Indian J Sex Transm Dis. 2006;27(1):24.
First Aid 2014 page 141, 467]]

Approved Approved::No
Keyword WBRKeyword::Tabes dorsalis, WBRKeyword::Syphilis, WBRKeyword::Dorsal columns, WBRKeyword::Posterior columns, WBRKeyword::Proprioception, WBRKeyword::Pressure, WBRKeyword::Vibration, WBRKeyword::Touch, WBRKeyword::Romberg, WBRKeyword::Argyll-Robertson pupil, WBRKeyword::Argyll, WBRKeyword::Robertson, WBRKeyword::Pupil, WBRKeyword::Pupils
Linked Question Linked::
Order in Linked Questions LinkedOrder::