An atypical presentation of paraneoplastic syndrome in lung cancer

1. Dr. Jheelam BiswasDr. Jheelam Biswas
Resident Medical OfficerResident Medical Officer
Neurology Unit-IINeurology Unit-II
THUESDAYTHUESDAY
CASE PRESENTATIONCASE PRESENTATION

2. Presenting complaintsPresenting complaints
Mr. Y, 47 year old diabetic gentleman, hailingMr. Y, 47 year old diabetic gentleman, hailing
from Mohammadpur, Dhaka was admitted underfrom Mohammadpur, Dhaka was admitted under
Dept of Neurology Unit II of this hospital on 6Dept of Neurology Unit II of this hospital on 6thth
October 2014 with the complaints of:October 2014 with the complaints of:
 Weakness of right side of body for ten daysWeakness of right side of body for ten days
 Difficulty in swallowing for ten daysDifficulty in swallowing for ten days

3. History of present illnessHistory of present illness
According to the statement of the patient, heAccording to the statement of the patient, he
was reasonably well ten days back. Then hewas reasonably well ten days back. Then he
experienced sudden weakness in the right side of hisexperienced sudden weakness in the right side of his
body. It was accompanied by difficulty in swallowingbody. It was accompanied by difficulty in swallowing
liquid food.liquid food.
With these complaints he sought admission inWith these complaints he sought admission in
BIRDEM Hospital for further evaluation andBIRDEM Hospital for further evaluation and
management .management .

4. History of present illnessHistory of present illness
On further query, the patient stated that he hadOn further query, the patient stated that he had
first noticed weakness in his lower limbs, morefirst noticed weakness in his lower limbs, more
marked in right since the beginning of Septembermarked in right since the beginning of September
2014. The weakness was gradually progressive and2014. The weakness was gradually progressive and
noticeable while walking and climbing stairs.noticeable while walking and climbing stairs.

5. History of present illnessHistory of present illness
HHe also gave history of weakness and heaviness ofe also gave history of weakness and heaviness of
both arms which was associated with occasionalboth arms which was associated with occasional
lack of ability while continuing to wash himself.lack of ability while continuing to wash himself.
The patient experienced infrequent episodes ofThe patient experienced infrequent episodes of
coughing and chocking while swallowing solid foodcoughing and chocking while swallowing solid food
and also liquids during the past one month .and also liquids during the past one month .

6. He suffered from weight loss during for theHe suffered from weight loss during for the
past six months but had no change in bowel andpast six months but had no change in bowel and
bladder functions, muscle twitching and gave nobladder functions, muscle twitching and gave no
preceding history of anorexia , fever, cough orpreceding history of anorexia , fever, cough or
hemoptysis .hemoptysis .
History of present illnessHistory of present illness

7. Past historyPast history
CAD (H/O PCI to LCX in 2010)CAD (H/O PCI to LCX in 2010)
Personal historyPersonal history
Ex-Smoker (till 2010) for 15 pack yearEx-Smoker (till 2010) for 15 pack year
Family historyFamily history
Nothing contributory .Nothing contributory .

8. Socioeconomic conditionSocioeconomic condition
Middle class familyMiddle class family
Drug historyDrug history
• T. Aspirin (75mg),T. Aspirin (75mg),
• T. Bisoprolol (5mg),T. Bisoprolol (5mg),
• T. Metformin (500mg)T. Metformin (500mg)

9. General examinationGeneral examination
Appearance: ill lookingAppearance: ill looking
Body built: Below avgBody built: Below avg
Height:Height:
Weight:Weight:
Decubitus: on choiceDecubitus: on choice
Co-operation: Co-operativeCo-operation: Co-operative
Anaemia:Anaemia:
Jaundice:Jaundice:
Cyanosis:Cyanosis:
Clubbing:Clubbing:
Leukonychia : AbsentLeukonychia : Absent
Koilonychias:Koilonychias:
Odema:Odema:
Dehydration:Dehydration:
Lymph node: Not PalpableLymph node: Not Palpable

10. General examinationGeneral examination
Thyroid gland: NotThyroid gland: Not
enlargedenlarged
Neck vein: Not engorged.Neck vein: Not engorged.
Hair distribution: NormalHair distribution: Normal
Skin condition: NormalSkin condition: Normal
Bed side urine: NADBed side urine: NAD
Pulse: 76/minPulse: 76/min
BP:140/80 mmHgBP:140/80 mmHg
Resp. rate:16/minResp. rate:16/min
Temperature: 98 FTemperature: 98 F

11. Nervous system examinationNervous system examination
Higher psychic function –Higher psychic function –
Dysarthria with nasal quality of speechDysarthria with nasal quality of speech

12. Nervous system examinationNervous system examination
Cranial nerves examination –Cranial nerves examination –
• Partial third nerve palsy of right with preservation ofPartial third nerve palsy of right with preservation of
pupillary size and reflex.pupillary size and reflex.
• Lower motor facial palsy on rightLower motor facial palsy on right
• IX and X palsy as evidenced by palatal paralysis andIX and X palsy as evidenced by palatal paralysis and
dysphagia to liquids with depressed coughdysphagia to liquids with depressed cough

13. Nervous system examinationNervous system examination
Cranial nerves examination –Cranial nerves examination –
• Partial third nerve palsy of right.Partial third nerve palsy of right.
• Lower motor facial palsy on right .Lower motor facial palsy on right .
• IX and X palsy present.IX and X palsy present.

14. Nervous system examinationNervous system examination

15. Motor System examination
MuscleMuscle
Rt. ULRt. UL Lt. ULLt. UL Rt. LLRt. LL Lt. LLLt. LL
BulkBulk
NN NN NN NN
ToneTone NN NN NN NN
PowerPower 4/54/5 4/54/5 4/54/5 4/54/5
InvoluntaryInvoluntary
movementmovement
AbsentAbsent AbsentAbsent AbsentAbsent AbsentAbsent

16. ReflexesReflexes BB TT SS KK AA AbdAbd
PlantarPlantar
ResponseResponse
RightRight ++++++ ++++ ++++ ++++++ ++++ NN EquivocalEquivocal
LeftLeft
++++++ ++++ ++++ ++++++
++++ NN
EquivocalEquivocal
Motor system examinationMotor system examination

17. Nervous system examinationNervous system examination
Sensory system: IntactSensory system: Intact
Signs of Meningeal irritation: Absent.Signs of Meningeal irritation: Absent.
Cerebellar signs : AbsentCerebellar signs : Absent
Romberg's test : Could not be evaluatedRomberg's test : Could not be evaluated
GaitGait : Could not be evaluated: Could not be evaluated

18. Local ExaminationLocal Examination
A hard, non tender swelling of about 5x5 cm inA hard, non tender swelling of about 5x5 cm in
size was present over sternum which was fixed tosize was present over sternum which was fixed to
underlying structure. Overlying skin was normal.underlying structure. Overlying skin was normal.

19. Respiratory System ExaminationRespiratory System Examination
• Inspection: Chest movement was symmetrical.Inspection: Chest movement was symmetrical.
• Palpation: Trachea was in central position and apexPalpation: Trachea was in central position and apex
beat was present in left 5beat was present in left 5thth
ICS. Chest expansion wasICS. Chest expansion was
symmetrical.symmetrical.
• Percussion: Percussion note dull on left .Percussion: Percussion note dull on left .
• Auscultation: Breath sound was diminished from leftAuscultation: Breath sound was diminished from left
lower zone. No added sounds were present.lower zone. No added sounds were present.

20. Other Systemic ExaminationsOther Systemic Examinations
• Musculoskeletal system : NADMusculoskeletal system : NAD
• Alimentary systemAlimentary system : NAD: NAD
• Cardiovascular system: NADCardiovascular system: NAD
• Genitourinary systemGenitourinary system : NAD: NAD
• Gynaecomastia and testicular atrophy ( absent)absent)

21. Salient FeaturesSalient Features
Mr. Y, a 47 years old, diabetic, hypertensive, ex-Mr. Y, a 47 years old, diabetic, hypertensive, ex-
smoker, was admitted under Neurology Unit 2 with thesmoker, was admitted under Neurology Unit 2 with the
complaints of sudden weakness in his right side andcomplaints of sudden weakness in his right side and
difficulty in swallowing of liquid foods for 10 days. Hisdifficulty in swallowing of liquid foods for 10 days. His
complaints were preceded by intermittent andcomplaints were preceded by intermittent and
progressive weakness in his lower limbs moreprogressive weakness in his lower limbs more

22. Salient Features (cont)Salient Features (cont)
noticeable during walking and climbing stairs, and innoticeable during walking and climbing stairs, and in
upper limbs during washing himself for 1 month. Heupper limbs during washing himself for 1 month. He
also had intermittent swallowing difficulty to both solidalso had intermittent swallowing difficulty to both solid
and liquid foods. He had history of PCI in 2006.and liquid foods. He had history of PCI in 2006.
On examination, the patient was ill looking, and his bodyOn examination, the patient was ill looking, and his body
built was below average. His vitals were in normalbuilt was below average. His vitals were in normal

23. Salient Features (cont)Salient Features (cont)
limits. On neurological examination, he had dysarthialimits. On neurological examination, he had dysarthia
with nasal speech, partial 3with nasal speech, partial 3rdrd
nerve palsy and lowernerve palsy and lower
motor type of 7motor type of 7th,th,
99thth
and 10and 10thth
nerve palsy. Bulk and tonenerve palsy. Bulk and tone
of muscles of all four limbs were normal, muscle powerof muscles of all four limbs were normal, muscle power
4/5, deep tendon reflexes were exaggerated in both4/5, deep tendon reflexes were exaggerated in both
biceps and knee, planter reflexes bilaterally equivocal,biceps and knee, planter reflexes bilaterally equivocal,

24. Salient Features (cont)Salient Features (cont)
muscle fasciculation was absent. A hard, non tender,muscle fasciculation was absent. A hard, non tender,
immobile swelling was noted over sternum. Diminishedimmobile swelling was noted over sternum. Diminished
breath sound and dull percussion note was present inbreath sound and dull percussion note was present in
lower left lung field. All other systemic examinationlower left lung field. All other systemic examination
revealed no abnormalities.revealed no abnormalities.

25. Provisional DiagnosisProvisional Diagnosis
• ICSOL in /around brainstemICSOL in /around brainstem
• DMDM
• HTNHTN
• CAD (S/P PCI to LCX)CAD (S/P PCI to LCX)

26. Differential diagnosisDifferential diagnosis
• Progressive bulbar palsy (variant ofProgressive bulbar palsy (variant of
Motor neuron disease)Motor neuron disease)
• SyrigobulbiaSyrigobulbia
• Myasthenia GravisMyasthenia Gravis
• Paraneoplastic neurological syndromeParaneoplastic neurological syndrome

27. Investigations
CBCCBC
• Hb% - 12.9 gm/dlHb% - 12.9 gm/dl
• WBC- 7600/ cummWBC- 7600/ cumm
Neutrophil- 66%Neutrophil- 66%
Lymphocyte-30%Lymphocyte-30%
Monocyte- 1%Monocyte- 1%
• Platelets- 2,34000/ cummPlatelets- 2,34000/ cumm
• ESR- 40 mm in 1ESR- 40 mm in 1stst
hrhr

28. Urine R/M/EUrine R/M/E
• Pus cells – 0-2/ HPFPus cells – 0-2/ HPF
• Epithelial cells- 2-3/ HPFEpithelial cells- 2-3/ HPF
• Sugar- NilSugar- Nil
• Albumin- TraceAlbumin- Trace
• Acetone- +Acetone- +

29. RFTRFT
S. Creatinine: 0.8mg/dlS. Creatinine: 0.8mg/dl
S. Urea: 28 mg/dlS. Urea: 28 mg/dl
LFTLFT
AST -24u/lAST -24u/l
ALT - 18 u/lALT - 18 u/l
ALP- 72 u/lALP- 72 u/l
S. Bilirubin - 0.9mg/dlS. Bilirubin - 0.9mg/dl
S. Albumin- 47.7gm/dLS. Albumin- 47.7gm/dL
S. Total Protein - 75.7 gm/dLS. Total Protein - 75.7 gm/dL

30. S.Electrolytes
• Na- 136 mmo/lNa- 136 mmo/l
• K- 3.6 mmol/lK- 3.6 mmol/l
• Cl- 95 mm0l/lCl- 95 mm0l/l
• Co2- 25 mmol/lCo2- 25 mmol/l
• Ca- 9.2 mmol/lCa- 9.2 mmol/l
• Mg- 0.8 mm0l/lMg- 0.8 mm0l/l

31. Lipid ProfileLipid Profile
• S. Chol- 120 mg/dlS. Chol- 120 mg/dl
• TG- 52 mg/dlTG- 52 mg/dl
• HDL-38 mg/dlHDL-38 mg/dl
• LDL- 71 mg/dlLDL- 71 mg/dl
Sugar ProfileSugar Profile
• FBS- 6.1 mmol/lFBS- 6.1 mmol/l
• ABF – 9.7 mmol/lABF – 9.7 mmol/l
• AL- 6.4 mmol/lAL- 6.4 mmol/l
• AD- 9.3 mmol/lAD- 9.3 mmol/l
• HbA1C- 6.1%HbA1C- 6.1%

32. ECGECG
No abnormality detectedNo abnormality detected

33. CXR P/A view :CXR P/A view :7.10.14
Consolidation in left upper and middle zone.Consolidation in left upper and middle zone.

34. MRI of brain
No abnormality detected.

35. MRI of brain

36. MRI of brain

37. MRI of cervical spine :MRI of cervical spine :
Degenerative changes. Thecal sac
indentation and bilateral neural foramina
narrowing at C3-4 level

38. MRI of cervical spine:MRI of cervical spine:

39. MRI of cervical spineMRI of cervical spine

40. NCV crossed limbs and EMG:10.12.14NCV crossed limbs and EMG:10.12.14
•Asymmetrical motor axonopathy.Asymmetrical motor axonopathy.
•Denervation of few muscles of lower limbs and tongueDenervation of few muscles of lower limbs and tongue
without spontaneous activities.without spontaneous activities.

41. RNS of ulnar and facial nerves

42. RNS of ulnar and facial nerves

46. FNAC from anterior chest wall lesionFNAC from anterior chest wall lesion
L/P/F L/P/F

47. FNAC from anterior chest wall lesionFNAC from anterior chest wall lesion
H/P/F H/P/F

48. Histopathology report
Smear shows anaplastic cells having scantySmear shows anaplastic cells having scanty
cytoplasm, round to oval hyperchromatic nucleicytoplasm, round to oval hyperchromatic nuclei
with coarse chromatin and inconspicuous nucleoli.with coarse chromatin and inconspicuous nucleoli.
They are arranged in clusters, rosettes and singly.They are arranged in clusters, rosettes and singly.
Many lymphocytes and histiocytes are seen in theMany lymphocytes and histiocytes are seen in the
background of blood.background of blood.

49. • Impression:Impression: FNAC from anterior chest wallFNAC from anterior chest wall
• Positive for malignant cells. Compatible withPositive for malignant cells. Compatible with
metastatic small cell carcinoma of lung.metastatic small cell carcinoma of lung.

50. CXR P/A view :9CXR P/A view :9.10.14

51. CT Scan ofCT Scan of chest 12.10.14chest 12.10.14
Right para tracheal
lyphadenopathy
Broad based soft tissue mass
in left anterior lateral chest
wall

52. CT Scan ofCT Scan of chestchest
Right para tracheal
lymphadenopathy
Mildly enhancing soft tissue
mass in left anterior lateral
chest wall

53. CT Scan of chestCT Scan of chest
post contrast post contrast

54. CT Scan of chestCT Scan of chest
• Impression-Impression-
• Suggestive of consolidation in left hilar regionSuggestive of consolidation in left hilar region
(? Primary) with pleural base soft tissue mass in left(? Primary) with pleural base soft tissue mass in left
para vertebral , left anterior chest wall, anteriorpara vertebral , left anterior chest wall, anterior
mediastinum, extending upto chest wall in left paramediastinum, extending upto chest wall in left para
sternal region and right para trachealsternal region and right para tracheal
lymphadenopathy.lymphadenopathy.

55. Specialist consultationSpecialist consultation
• Specialist consultation was taken from anSpecialist consultation was taken from an
oncologist from BSMMU, and was advised to do theoncologist from BSMMU, and was advised to do the
following investigations prior to specific approach-following investigations prior to specific approach-
• CT guided FNAC from lung lesionCT guided FNAC from lung lesion
• EchocardiogramEchocardiogram
• Tumor markers- CEA, PSA, CA 19.9Tumor markers- CEA, PSA, CA 19.9
• Whole body bone scanWhole body bone scan

56. Tumor MarkersTumor Markers
• CEA- 0.73CEA- 0.73
• CA 19-9- 10.8CA 19-9- 10.8
• CA- 125- 12.4CA- 125- 12.4
• PSA- 16.3PSA- 16.3

57. Final DiagnosisFinal Diagnosis
Small cell carcinoma of lungSmall cell carcinoma of lung
Myasthenia GravisMyasthenia Gravis
DMDM
HTNHTN
CAD (S/P PCI to LCX)CAD (S/P PCI to LCX)
Axonal neuropathyAxonal neuropathy

58. TreatmentsTreatments
• Diet control for diabetesDiet control for diabetes
• Aspirin 75 mg 0+1+0Aspirin 75 mg 0+1+0
• Bisoprolol 5mg 1+0+0Bisoprolol 5mg 1+0+0
• Losertan 25 mg 0+0+1Losertan 25 mg 0+0+1
• Pyridostigmine 60mg 1/2+ 1/2+ 1/2Pyridostigmine 60mg 1/2+ 1/2+ 1/2
• PhysiotherapyPhysiotherapy

59. Definite ManagementsDefinite Managements
• Natural history and prognosis was explained toNatural history and prognosis was explained to
patientpatient
• Plan : Chemotherapy- 6 cycles.Plan : Chemotherapy- 6 cycles.

60. Discussion
• The relationship between presynaptic
neuromuscular junction (NMJ) disorder (Lambert-
Eaton myasthenic syndrome) and malignant tumors
has long been recognized.
• But the association of extrathymic malignancies
with myasthenia gravis (MG) is an attractive topic.

61. • MG is considered as a paraneoplastic syndromeMG is considered as a paraneoplastic syndrome
associated with thymoma in 15% of MG patients.associated with thymoma in 15% of MG patients.
• Extrathymic malignancies have been also reportedExtrathymic malignancies have been also reported
to happen simultaneously with MG.to happen simultaneously with MG.

62. • To date, no one has found causal relationshipTo date, no one has found causal relationship
between lung cancer and MG.between lung cancer and MG.
• Similarly, there has been no supporting evidenceSimilarly, there has been no supporting evidence
that simultaneous MG with lung cancer might be onethat simultaneous MG with lung cancer might be one
of the paraneoplastic syndromes such as Lambert-of the paraneoplastic syndromes such as Lambert-
Eaton myasthenic syndrome with small cell lungEaton myasthenic syndrome with small cell lung
cancer.cancer.

63. • In conclusion, this case was a typical MG but hisIn conclusion, this case was a typical MG but his
symptoms were the only clinical presentation of hissymptoms were the only clinical presentation of his
underlying lung cancer.underlying lung cancer.