This case presentation summarizes a 55-year-old man with a 1-year history of tingling and itching sensations ascending from his face down his body. Examination found hyperalgesia everywhere except below the knees. Differential diagnosis includes non-length dependent small fiber neuropathy potentially due to diabetes, hepatitis C, or autoimmune disorder. Imaging found hepatic lesions concerning for malignancy. Further workup includes additional autoantibody testing, HCV viral load, CT scan, and skin biopsy.

1. TUESDAY GRAND ROUND
CASE PRESENTATION
Yasser A. Alzainy
Mohamed Hamdy Basiony
5/9/2023
AL-AZHAR UNIVERSITY
Faculty of Medicine
Neurology Department

2. PERSONAL HISTORY
• A fifty-five-year-old man, form Ain-Shams District, Cairo (born in
Shubra). He is right-handed and used to work as financial manager
then retired in 2016 and now on pension.

3. COMPLAINT
• Tingling sensation and itching all over the body.

4. HISTORY OF PRESENT ILLNESS
• The patient's condition has started one year ago by insidious onset
and progressive course of tingling and itching sensation all over the
face.
• The patient sought dermatological advice, diagnosed as dysesthesia,
and was treated with Gaptin (Gabapentin) 300mg qhs and Betallerge
(Betamethasone 0.25 mg+ Dexchlorpheniramine Maleate) tab bid;
with moderate improvement of symptoms for about 4 weeks.

5. • One month later, the patient's symptoms progressed, to encompass
both thighs with progressive ascending march that involved the
whole trunk along 2 months period.
• In the subsequent months, the symptoms intensified, with
sensations of tingling, pins and needles, and burning becoming more
pronounced. These discomforting sensations could now be triggered
by even gentle touch and clothing, and their severity heightened.
• Notably, the regions below the knee remained unaffected up to the
present moment.

6. • The condition was associated with gradual thinning / loss of body hair
over the course of illness.
• The patient also reports unintentional loss of weight (about 6 kg over
2 months).
• Both manifestations were attributed by his physician to poor
glycemic control.
• The patient developed multiple painless subcutaneous swellings in
the left supraclavicular region and right arm.

7. • There is no history of suggestive of motor or autonomic dysfunction.
• No history suggestive of cranial nerve affection or cerebellar
manifestations.
• No history suggestive of cognitive impairment, behavioral changes or
encephalopathy.
• There is no history suggestive of other systems affection (arthritis,
renal impairment, respiratory problems, ocular inflammation, sicca
syndrome, etc.).

8. PAST HISTORY
• Diabetes Mellitus
o Diagnosed in 2008
o Poor control
• HCV +ve
o Diagnosed in 2006
o Treated with Sovaldi in 2016 (successful).

9. • No history of previous hospital admission,
surgical intervention or blood transfusion
• No history of substance use.

10. SOCIAL AND TRAVEL HISTORY
• The patient is married and has one daughter (17 years old).
• No special habits of medical importance.
• Travelled to work in KSA from (1997) to (2016)

11. FAMILY HISTORY
• Unremarkable
 No history of similar condition.
 No consanguinity.

12. FORMULATION
• A 55-yo male patient who is diabetic and HCV+ve presenting with gradual onset
progressive course of:
• Ascending dysethesia with sparing of regions below knees
• Thinning of hair
• Significant weight loss
• ?Lymphadenopathy

13. DIFFERENTIAL DIAGNOSIS

14. WHERE IS THE LESION?
 Generalized
 Symmetrical
 Non-length dependent
 Pure sensory (? Sensorimotor)
 Polyneuropathy
• Where is the lesion
• Systemic
• Peripheral nerves

16. POSSIBLE ETIOLOGY
• Non-length-dependent SFN (NLD-SFN)
• Diabetes mellitus
• HCV (CG +ve or –ve)
• Autoimmune disorder (Sarcoidosis; Sjogren Syndrome)
• Paraneoplastic
• Sensory neuronopathy
• Paraneoplastic
• Autoimmune (Sjogren syndrome / Idiopathic)

19. GENERAL EXAMINATION

20. • BP: 120/70 (no postural hypotension)
• Pulse: 76 beats/m, regular pulse.
• RR: 14 cycle/m.
• T: 37◦ C.
• Thin patient with mild pallor, no jaundice or cyanosis, swelling in supraclavicular
region and along the medial border of biceps, with no detectable
organomegaly.

21. • Head and Neck: no characteristic facies, normal thyroid.
• Chest: fair air entry, no crepitations or wheezes.
• Heart: normal S1 & S2, no murmurs, gallops or rubs.
• Abdomen: lax abdomen, not tender, no detectable ascites or
suprapubic dullness, no organomegaly.
• Skin : multiple papules with similar morphology on the chest and
lower limbs

23. NEUROLOGICAL EXAMINATION

24. MENTAL STATE
• The patient is fully conscious, attentive, oriented to time, place and
persons with euthymic mood, intact memory to recent and remote
events and she is cooperative.
• MMSE score = 30

25. SPEECH
• No Dysphasia or Dysarthria

26. CRANIAL NERVES
 Olfactory: intact
 Optic:
o Visual Acuity: Rt: 6/6; Lt: 6/6.
(corrected)
o Color vision: intact
o Visual field: intact
o Fundus examination: Bilateral
normal fundi
 Occulomotor, Trochlear,
Abducens: Intact.

27. CRANIAL NERVES
 Trigeminal nerve:
 Intact motor and sensory examination.
 Facial nerve:
 Intact.
 Glabellar reflex: normal
 Vestibulo-Cochlear nerve: intact.

28. CRANIAL NERVES
 Glossopharyngeal, Vagus:
 Uvula: Centralized
 Palatal movement: Intact on both side
 Palatal & Pharyngeal Reflexes: present bilaterally
 Hypoglossal Nerve:
 Tongue: no wasting, abnormal movements or fasciculation.

29. MOTOR SYSTEM
 Muscle State: no wasting, fasciculations or tenderness.
 Muscle Tone: Normotonia.
 Power: Grade 5 throughout

30. REFLEXES
 Deep Reflexes: Normoreflexia except
ankle reflex (hyporeflexia).
 Superficial Reflexes
 Abdominal: Present bilaterally (upper, middle, lower)
 Plantar: bilateral flexor plantar.
 No pathological reflexes could be elicited
2+
1+
2+ 2+
2+ 2+
2+
2+
2+
1+

31. SENSORY SYSTEM
 Superficial:
 Pain: hyperalgesia all over the body except from knee downwards
 Touch: intact
 Deep: intact vibration and position sense.
 Cortical: intact.

32. CEREBELLUM
• No ataxia on various cerebellar tests.

33. BACK
 No scoliosis or kyphosis.
 No skin pigmentation.
 No localized tenderness.

34. GAIT
• Average stance, pace; within normal base and arm swing

35. STRETCH SIGNS AND ROMBERG’S SIGN
 Negative.

36. LABORATORY TESTS
• ESR= 70 (1st hour)
• Hb = 12 gm/dl
• TLC = 5.7 k/ul (lymph = 1.2 k/ul)
• PLT = 320 k / ul
• KFT: within normal
• ALT = 113
• AST = 87
• Albumin=2.6
• INR = 1
• ANA, RF, Anti-DNA: pending
• C3 and C4: pending
• Bence jones proteins : NAD
• Serum IgE =149.9 k / l
• Hba1c=13.8
• Alpha fetoprotein = 141 (up to 8)

37. LABORATORY TESTS
• Serum Protein Electrophoresis:
• Hypoalbuminemia
• Increased Beta 1 fraction &
polyclonal
hypergammaglobulinemia.
• Kappa light chain: increased
• Lambda light chain: increased
• Kappa / Lambda = normal.
• Immune fixation:
• 2 heavy chain bands at IgG and
IgA fractions.
• 2 light chain bands at Kappa and
Lambda regions.

38. NEUROPHYSIOLOGY

39. NCS OF BOTH UL AND LL
• Axonal neuropathy of both peroneal nerves.
• Otherwise, unremarkable

40. IMAGING
• Pelvi-abdominal U/S:
• Right hepatic focal lesion measuring 8.7 x 8.6 cm
• Left hepatic focal lesion measuring 5.6 x 6.3 cm

41. SUGGESTED PLAN
• Labs:
o Anti-Ro, Anti-La
o HIV
o HCV PCR
• Imaging:
o Triphasic CT (Abdomen)
• Biopsy:
o Skin biopsy for IENFD

42. Small Fiber Neuropathy - Practical Neurology