A rare neurological case

1. INTERESTING CASE
PRESENTATION

2. • 53/M
• Kozhikkode
• Security officer

3. Presenting illness
Progressive episodic stiffness and painful
spasms of body and both legs – 2 years

4. History of presenting illness
• His symptoms began insidiously in the form of
episodic and progressive stiffness and pain in the
entire back which became worse with tension or
stress.
• He had brief episodes of dramatic severe
worsening that resolved spontaneously.
• One year ago he started developing stiffness of
thigh and arms, particularly when he heard loud
noises , changing tv channels , cold wind blowing
on his body , became angry, or frightened.

5. • This sort of stimulus evoked painful severe
spasms that resolved slowly. He began to move
very slowly because rapid movement induced
severe spasms. Even the distal extremities may
become involved when he moved rapidly.
• Slowly his quality of life became affected severely
, making it difficult for him to work.
• Slowly other simple activities of daily living
became problematic and didn’t have a satisfying
social life.

6. • No h/o stiffness of facial muscles ,weakness of
limbs , sensory impairment , ataxia ,abnormal
body movements , visual disturbance , facial
weakness , difficulty swallowing , dysarthria ,
seizure , memory impairment , loc , bowel
bladder disturbance , trismus , fever .

7. Past history
• No known co-morbidities .
• No history of similar illness in the past . No
h/o any significant medical or surgical illness
in the past.

8. Family history
• No h/o similar illness in the family .
• No f/h/o neurological illnesses in the family .

9. Personal history
• Smokes 8 to 10 cigarattes daily for the past 20
years .
• Non alcoholic
• Bowel , bladder normal .
• Sleep adequate .
• Consumes a mixed diet

10. Socio economic history
• Middle class family .

11. • To summarise …

12. General examination
• Conscious , cooperative .
• Sitting position in bed.
• Normally built and nourished .
• No
Pallour/Icterus/cyanosis/clubbing/lne/edema .
• BP : 130/90 mmHg ; PR : 86/mt RR: 16/mt
• T:Afebrile ;
• Skin, hair , nails - normal

13. CNS
• Hmf – Normal
• Cranial Nerves – Normal
• Motor system
bulk – normal
Power – normal
tone –hypertonia b/l ul + ll
reflexes – dtr – brisk b/l .
superficial reflex - normal

14. • Sensory – normal
• Cerebellar system – normal
• Gait – cautious gait with decreased arm swing.
No signs of meningeal irritation .
Skull and spine normal.

15. Other systems
• Cvs – S1 S2 heard . No murmur.
• RS – AEBE , NVBS b/l . No added sounds.
• P/A – soft ; No hsm .

16. Differential diagnosis .…?
• Tetanus.
• Painful tonic spasms of multiple sclerosis.
• Idiopathic hyperekplexia.
• Somatoform disorder.
• Myoclonic seizures.
• Axial torsion dystonia.
• Neuromyotonia (Isaac syndrome).
• Moersch Woltmann Syndrome .

17. investigations
• Blood r/e
• Wbc-6000
• N68;L22:E0;M0
• ESR-22
• Hb-14
• Platelet-225

18. • LFT, RFT, SE, S.Ca2+, S.Mg2+,S.K+ , S.Ph– Normal
• Ur/e , S.Uric Acid , Rbs ,Ecg – Normal
• ANA – Negative , CRP – normal
• TSH,FT4 – Normal .
• Blood c/s – Normal
• MRI Brain with Whole spine screening – wnl
• CSF study - Normal

19. • Chest x ray

20. Plain CT -Thorax

21. Ct Guided FNAC of Lesion
• s/o Adenocarcinoma Lung .

22. Review the diagnosis
• Is that a Paraneoplastic syndrome ????

23. Moersch Woltmann Syndrome
Or
Stiff Man Syndrome

24. INTRODUCTION
• Rare neurologic disease with lack of significant
similarity to any other neurologic diseases.
• Once seen it is quite unforgettable.
• Closest related disease is tetanus .
• Associated with a number of non-neurologic
diseases, mainly DM and thyroiditis.
• One in ten lakh population .

25. Pathophysiology
• Unclear

27. Clinical presentation˜˜˜˜˜

36. DIAGNOSIS
• Stiffman Syndrome
• Neurologic Paraneoplastic manifestation of
Adenocarcinoma lung .

37. Thank you .