This document summarizes a case of a patient presenting with intermittent shaking of the lower extremities. After examination and testing, the patient was diagnosed with systemic sarcoidosis involving the eyes, lungs, and central nervous system. Key findings included chorioretinal vasculitis, hilar lymphadenopathy, and elevated ACE levels. MRI of the brain was unremarkable. The patient was discharged on a steroid taper and follow up with rheumatology given the multisystem involvement and prognosis of potential permanent damage requiring long term management.

1. Naresh Mullaguri MD
Resident Physician
Department of
Neurology
University of Missouri

2. Chief complaint
Intermittent shaking of bilateral lower extremities

3. HISTORY OF PRESENT ILLNESS

5. PERTINENT HISTORIES
PMH: Paranoid Schizophrenia in 2010 but not being
following up with Psychiatry. Mentioned that he is not
hearing any voices lately
Ranitidine and Promethazine by his PCP
NKDA
Single, Finished 10th grade, works at a restaurant and
denies drinking, Quit smoking 1 week ago but used to
smoke ½ - 1 PPD, Denied recreational drug use, Denied
any unprotected sexual intercourse or STI in the past.
Migraines in mother, Maternal grand mother and great
grandmother had Nervous break downs, Don’t know any
history from the paternal side. Positive for DM, Cancer,
HTN and CAD

6. MY FLOW OF THOUGHT
?Dealing with a systemic disease with focal
neurological symptoms
Sounds like demyelinating diseases like MS or NMO
– age of presentation and involvement of CN II and a
ton of other constitutional symptoms
I miss HIV, Tuberculosis but its counterpart
Sarcoidosis, Lyme’s, opportunistic infections here in
the US and he is an African American
? May be an unfortunate guy with
cancer/Autoimmune issues

7. Lets go to exam
Vital signs:
Temperature: 37.2
PR: 85, Regular
RR: 16
BP: 110-120/70-80s
BMI: 24 (Weight loss from 210 – 188 pounds)

8. General: Well dressed, well spoken African American male,
looks appropriate for the age. Not in distress
HENT: Atraumatic, no rash or vesicles noticed, Oral mucosa is
moist, external auditory canals were patent and normal. No
tonsillar hypertrophy or pharyngeal wall erythema, No PNS
tenderness. No thyromegaly, No wasting of facial muscles.
Respiratory system: Non labored breathing, No wheezes, Clear
Cardiovascular System: S1 and S2 heard, No pedal edema
Gastrointestinal: Soft, no tenderness or organomegaly palpated
Musculoskeletal: No joint effusion, tenderness or wasting.
Integument: No rash, clubbing

9. Neurological:
Higher Mental functions:
Cranial Nerves:
Dilated
fundus exam revealed edematous optic disc, retinal Vasculitis, Uveitis and
crystalline deposits in the vitreous. Unable to confirm Papilledema
Motor exam:
Sensory exam:
Coordination:

10. Differential Diagnosis
Optic neuritis/Papillitis secondary to HIV, Sarcoidosis,
Tuberculosis, Lyme’s disease, syphilis or other
opportunistic infections
Multiple Sclerosis/Neuromyelitis and other autoimmune
diseases like Behcet’s disease, Lupus and Sjogren
syndrome.
CNS Vasculitis/Autoimmune Vasculitis (systemic like PAN)
Lymphoma or other forms of cancer

11. INVESTIGATIONS
Normal CBC
Normal BMP
ALT – 57
ESR – 21
Flow cytometry – Negative
Normal Thyroid profile
CSF : clear and colorless, Protein – 84, Glucose – 47,
WBC – 46 (93% Lymphocytes), RBC – 7, Lactic Acid –
1.7
UA is clean

12. MBP – 2.04
ACE level – High at 64 (Upper normal – 53)
Bartonella henselae titres elevated
MS profile Negative
HIV – Non reactive
Histoplasma, Blastomyces, Aspergillus, coccidioidomycosis, Toxoplasmosis, Toxocara,
Lyme’s serology, Crypto, VDRL in CSF were negative, Vasculitis and autoimmune panel
is negative.
Hepatitis panel is negative, Protein electrophoresis is negative
CSF culture, Blood cultures were negative.

13. IMAGING
FINDINGSMRI of the Brain – Both Orbit and Optic nerves are normal
No infarction, Midline structures were unremarkable. Venous
anomaly in the pons but otherwise unremarkable pre and post
contrast study.
MRA of the Head and Neck is unremarkable to check
vasculitis.
Chest Xray is normal with no hilar prominence
Chest CT showed Mediastinal and Bilateral hilar confluent
Lymphadenopathy with differential including Granulomatous
disease, Lymphoma and metastatic disease
Subcarinal Lymph node fine needle aspirate : No malignant

14. DIAGNOSIS
SYSTEMIC SARCOIDOSIS
CANDLE WAX APPERANCE OF
CHORIORETINAL VASCULITIS

15. CHORIORETINITS

16. Pulmonary involvement – almost always
Honey combing of Lung

17. HILAR LYMPHADENOPATHY

18. Non Caseating granuloma – Schaumann body

19. Neurosarcoidosis
Sarcoidosis is a multistep disease characterized by granulomatous
inflammation.
5-15% of sarcoidosis patient have CNS involvement. Involves both CNS
and PNS.
Symptoms including Headaches, Visual impairment, diplopia, ataxia,
motor deficits, seizures, cognitive decline. Ocular involvement typically
presents as Uveitis and rarely as orbital involvement affecting EOM and
lacrimal glands.

20. Cranial Neuropathies
Meningeal disease: Aseptic Meningitis, Mass lesion
Hydrocephalus
Brain disease: Endocrinopathy, Encephalopathy,
micro vasculopathy, Seizures, stroke, vegetative
dysfunction, extra or intramedullary spinal canal
disease, Cauda equina syndrome
Neuropathy: Mononeuropathy - axonal are
demyelinating, sensory/ motor/sensorimotor
Myopathy: Polymyositis, modules and atrophy
clinical presentation

21. CNS Sarcoidosis – Other symptoms to
review with patient
Menses, Libido, Galactorrhea
Excessive thirst – Osmostat, DI, Hyperglycemia,
Hypercalcemia, Hypercalciuria
Altered body temperature, sleep and appetite
LABS
TFTs(Hypothalamic Hypothyroidism), Prolactin,
Testosterone or Estradiol, FSH, LH and cortisol

22. Other Diagnosis to be considered
Multiple sclerosis
Neuromyelitis optica
Sjogren syndrome
Systemic lupus
erythematosus
Neurosyphilis
Neuroborreliosis
HIV
Lymphoma
Behcet’s disease
Vogt – Koyanagi – Harada
Toxoplasmosis
Brucellosis
• Whipple’s disease
• Germ cell tumors
• Craniopharyngioma
• Isolated CNS angitis
• Primary CNS neoplasia
• Lymphocytic Hypophysitis
• Pachymeningitis
• Rosai-Dorfman disease
• CMV Meningoencephalitis
• Low CSF pressure/volume and
Meningeal enhancement

23. IMAGING SPECTRUM
R. Shah et al. AJNR Am J Neuroradiology 2009;30:953-961

29. TREATMENT AND PROGNOSIS
Patient was discharged with oral taper of steroids
with follow up appointment with Rheumatology.
Spontaneous resolution in 4-6 months
Damage may be permanent.
Long term treatment is needed with Steroids and
steroid sparing drugs

30. THANK YOU
Photo taken by Naresh Mullaguri in Illinois from an
AMTRACK