Again, a conversion reaction !!!

 
   A 19 year old newly married female referred to us as a case of resistant "hysterical" lower limb weakness. She was completely healthy, reasonably well, self caring, and on no medications or drugs, no such a family condition, no risk factors for HIV, and no history of back trauma ,until 3 months ago when she started to drag her both feet while walking, which was attributed to "simple tiredness" at time. Her husband noticed that her gait became progressively difficult and she looked on to the ground while walking. No complaints regarding back pain, sphincters or sensations.

Her initial examination records documented an anxious young woman, poorly cooperative with the examiner; however, there was bilateral foot drop with absent ankles, equivocal planters, and ?stocking loss of all sensory modalities.  

  A battery of investigations was carried out including lab tests and EMG. The EMG and nerve conduction studies report was: " completely normal apart from poor recruitment, which could be to upper motor neuron lesion, or hysterical".

  Because she was anxious and uncooperative with examiner, together with the neurophysiology report, a diagnosis of a hysterical weakness was made and she was managed as such. Her condition unfortunately progressed to a degree of a total chair bound state.

  When we examined her, there was severe atrophy of the lower limbs muscles but no fasciculations, grade 1 distal and  grade 3 proximal asymmetric weaknesses, absent knees and ankles, equivocal planters, and stocking loss of all sensory modalities. With assistance to stand and walk, she showed a severe bilateral foot drop; Rombergism was positive.No sensory level at the trunk, and the upper limbs and cranials were normal.

  MRI of the lumbosacral area with contrast revealed a large irregular shaped mass inside the vertebral canal, with heterogenous enhancement. She was referred to neurosurgery; the neurosurgeon said that upon exploration, the mass was large, hard, and diffusely infiltrative causing bone destruction and cauda equina involvement; it was debulked only. Biopsy revealed a well differentiated adenocarcinoma of unknown primary site.

  We reviewed her general examination records and we repeated the clinical medical examination fully ; they were totally negative. Thorough investigations revealed a very small right-sided deep seated non-palpable breast cancer focus (with negative axillae) in the upper lateral quadrant.

 

Discussion:

  Metastatic tumor-related syndromes are well known to be one of the modes of presentation of a primary cancer. Her breast primary focus was asymptomatic and clinically silent and supple, and the presentation was  totally neurological in nature.

  This case highlights a common problem in the primary care settings; the over-diagnosis of hysterical conversion reactions. Please notice that a conversion reaction is a diagnosis of exclusion, and in the presence of SOLID signs indicating a RED flag, you should always be careful in the evaluation. Her past history indicates that she was healthy; bilateral foot drop is rarely (if ever) hysterical; and the pace of illness indicates that there is something there, grave and progressing, which definitely prompts a full search for a hidden pathology.

  She is now seen by an oncologist and is receiving chemotherapy and radiotherapy for a stage IV estrogen receptor negative breast adenocarcinoma.

 

Questions related to article No. 12:

 Q1: A 62 year old man developed right sided foot drop over 2 days with urinary retention. He is a known case of small cell lung cancer on no treatment since. In cauda equina lesions; which one is the correct statement?

a. The clinical findings are usually symmetrical.

b. Sacral anaesthesia is against the diagnosis.

c. Absent ankle reflex indicates a co-existent upper motor lesion.

d. Urinary retention is seen.

e. Back pain is always present.

Q2: A 38 year old homosexual man with advanced AIDS, on no anti HIV medications, presented with 1 week history of progressive lower limbs weakness and diffuse lancinating pains. A diagnosis of CMV necrotizing radiculitis of the cauda equina was made. Regarding clinical features of cauda equina lesions, which one is the correct statement?

a. Absent right ankle jerk and left anterior thigh anesthesia are against the diagnosis.

b. Sensory symptoms should be mild or absent.

c. Constipation is suggestive of co-existent pathology.

d. Diarrhea is very common.

e. Sensory araxia is seen.

Q3: A 17 year old previously healthy male presents with 2 week history of walking difficulty and urinary retention. He is on no medications including illicit drugs, no risk factors for HIV, and no history of back trauma. He did admit to the presence of strange numbness in his right thigh and left sole. Examination of the lower limbs  revealed right-sided foot drop, some wasting of the left thigh, grade 4 minus weakness proximally and grade 2 distally in the right ankle, absent left ankle and right knee jerks with equivocal planters, with loss of pinprick sensation in the left sole and right anterior thing. You detected large palpable lymph nodes the neck, axillae and groin with palpable spleen.

1)  All of the following investigations are of use, except:

a. Complete blood count.

b. Lymph node biopsy.

c. EMG.

d. Brain MRI with contrast.

e. Lumbosacral MRI with contrast.

 
2) What is the overall diagnosis?

a. Polymyositis and reactive lymphadenopathy.

b. Lumbosacral roots involvement by lymphoma.

c. Systemic lupus erythematosus and cerebral venous sinus thrombosis.

d. Hemispheric glioblastoma multiforme and meningeal drop metastases.

e. Multiple sclerosis and pseudolymphoma.

 
3) What is your next step?

a. Giving high dose pulse methyl prednisolone for 3 days only.

b. Whole brain irradiation.

c. Oncological consultation.

d. Plasma exchange.

e. Beta interferon injections.

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Q1:Answer: d

a. false, usually asymmetrical and confusing eg lost pinprick sensation in the right calf and left anterior thigh.

b. false, together with sphincteric problems are highly suggestive in the appropriate clinical setting.

c. false, a co-existent conus medullaris lesion might be seen as extensor planters; absent ankles indicates radicular lesion in cauda equina ( S1) involvement.

d. true. Together with asymmetric lower motor neuron and sensory signs in the lower limbs, should always prompt a search for a cauda equina pathology.

e false.

Q2: Answer: e

a. false, remember that the cauda equina is the lumbosacral roots gathered together in a narrow canal; so different roots could be involved.

b. false, could be absent, mild, severe, or the only presenting feature, depending the site of the lesion

c. false, Urinary retention and constipation could be prominent part of the clinical picture.

d. false, should suggest another diagnosis or a co-existent pathology.

e. true, due to involvement of large diameter fibers subserving kinesthesia.

Q3:Answers: d, b, c.

The overall picture is suggestive of lower motor neuron lesion at multiple sites, and cauda equina localization would fit. The presence of such large multiple lymph nodes and splenomegally may well point towards lymphoma as cause of this cauda equina syndrome. There are no focal hemispheric or upper motor neuron signs, so brain MRI is useless. Referring to an oncologist would be the correct final step in our management.

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