Acute Occipital Neuralgia secondary to Zoster. This is a case presentation of a rare secondary headache secondary to infection.

1. Naresh Mullaguri MD
Resident Physician PGY2
Department of Neurology
University of Missouri
A CASE OF ARNOLD’S NEURALGIA

2. ER CONSULT: EVALUATION OF SEIZURE LIKE ACTIVITY
HPI: 57 year old right handed Caucasian male patient presented for the third time to the ER with
severe left sided head and neck pain with associated yelling and whole body movements for the
past 7 days and has been having these episodes which last 1-2 minutes where he will get a burning
hot poker feeling in the back of his left neck that radiates up to his left scalp and partially into his left
shoulder. 10/10 in intensity. His daughter mentioned that during the spell, he will start crying, rolling
around in bed clutching his left side of the neck. He answers questions during the episodes and said
he was unable to stop doing these activities. No post ictal symptoms and he will be completely back
to baseline. The frequency is around 20 in 24 hrs. His spells are also associated with blurring of
vision in the left eye and feels like he has too many tears in the left eye. He had been evaluated in
the ER thrice for the same complaint. He mentioned he had shingles in the same distribution 3
weeks ago. He was prescribed Prednisone, Gabapentin, Amitriptyline and reports minimal to no
improvement. He will be free of any pain in between the spells.
He was prescribed Acyclovir for shingles at onset but he was unable to finish the course as he
developed abdominal pain and diagnosed as having cholecystitis after 2 days.
ROS: Denied any trauma, prior history of neck pain, migraines, similar complaints in the past,
Negative for other systems.

3. PAST MEDICAL /SURGICAL HISTORY:
TYPE 2 DIABETES
HYPERLIPIDEMIA
MOTOR VEHICLE ACCIDENT IN 1974 WITH CONCUSSION
HYPERTENSION
CHRONIC LOW BACK PAIN AND SURGERY
RIGHT ORCHIDECTOMY
SHINGLES 15 DAYS AGO and being treated for Post Herpetic Neuralgia
SOCIAL HISTORY:
LIVES WITH HIS WIFE, CURRENTLY WORKS IN FARM AND RETIRED AS A MECHANIC.
150 PACK YEAR SMOKING HISTORY BUT QUIT SINCE 2004, DENIED ALCOHOL AND
DRUGS.
FAMILY HISTORY:
MOTHER – BREAST CANCER
FATHER – BONE CANCER
GRANDMOTHER – DIABETES
NO KNOWN DRUG ALLERGIES

4. MEDICATIONS
• Lisinopril
• Atorvastatin
• Clement
Percocet 10/325 Q4H
• Lantus
• Baclofen
• Flexeril
• Gabapentin
• Aspirin
• Pantoprazole
• Valacyclovir
• Prednisone 50mg daily should be tapered in a week
• Amitriptyline

5. SUMMARY OF HISTORY
• 57 Y/O WHITE MALE PRESENTED TO THE ER MULTIPLE TIMES FOR THE
EVALUATION OF NEW ONSET EPISODES OF SEVERE LEFT SIDED HEAD AND NECK
PAIN AND HYPERMOTOR ACTIVITY WHICH STARTED 1 WEEK AFTER SHINGLES
AFFECTING THE SAME AREA. 1-2 MINUTE SPELLS WITH A FREQUENCY OF 20/DAY
• HISTORICAL DIFFERENTIAL DIAGNOSIS:
Occipital neuralgia
Hyper motor frontal lobe seizures
Referred pain to the Occipital region from Atlantoaxial joint or Zygapophyseal joints
Trigeminal Neuralgia

6. PHYSICAL EXAMINATION
Vitals: HR – 95, Resp. Rate: 20, NIBP – 119/79mm of Hg, Temp – 36.8, SpO2 – 92
General: moderately built and nourished white male in no distress between spells
Eyes: No corneal or conjunctiva lesions or erythema, no increased watering except during the
spells. Pupils were round, 4mm in diameter and reacting to light equally. Fundoscopic exam showed
sharp disc margins.
HENT : No rash is noticed, oral mucosa is moist and free of any lesions, external auditory canals
are patent and free of any vesicles, tympanic membranes are visible and clean. Slight tenderness in
the left occipital groove region but no dysesthesia over the scalp or over the temple. After my Head
and neck exam, within 2-3 minutes, he had another typical spell which lasted 2-3 minutes. No
tenderness over the cervical spines, mastoid regions or paranasal sinuses. No scars were visible on
the neck of prior shingles.
Respiratory: CTA bilaterally
Cardiovascular: Regular rate and rhythm without murmur
Gastrointestinal: soft, non-tender, non-distended
Musculoskeletal: no deformity, no edema
Psychiatric: cooperative, appropriate mood and affect.

7. NEUROLOGICAL EXAM
HIGHER MENTAL STATUS: awake, alert and oriented X 3, attention and concentration are
good. speech is fluent without dysarthria and comprehension is intact. Memory is intact to
recent and remote events.
CRANIAL NERVES: Visual fields are full to confrontation, EOM were intact, No facial sensory
loss to light touch or pin prick or asymmetry. Able to close his eyes, puff up his cheeks, Jaw
strength is normal. Strong voice, uvula is in midline and elevated symmetrically. SCM strength
is 5/5 bilaterally. Tongue protrudes to midline and moves sideways.
MOTOR EXAM: normal bulk and tone. Strength is 5/5 bilaterally in all the four extremities in
both proximal and distal groups. Gait is normal. Waling was a little difficult in th beginning due
to LP done in the ER. DTRs were 1+/4 in bilateral biceps, brachioradialis, triceps, knees and
ankles.
SENSORY EXAM: Intact to light touch and pinprick in all the areas. Vibration is intact with no
distal gradient. Plantars were down going bilaterally. Romberg’s sign is negative.
COORDINATION: finger to nose test and heel to shin test were normal. No truncal ataxia.
Able to perform rapid alternating movements.

8. CLINICAL DIFFERENTIAL DIAGNOSIS
• OCCIPITAL NEURALGIA
• Head or facial pain attributed to acute Herpes Zoster (HIS 13.15.1)
• Space occupying region compressing the Greater and Lesser Occipital nerves
• Frontal lobe seizures

9. INVESTIGATIONS
• Hb: 8.9
• RBC: 4.78
• HCT: 41.8%
• MCV: 87.4
• PLT: 251
• ESR: 9
• PT/PTT/INR – normal
• Na: 135
• K+ : 4.4
• Cl- 94
• CO2 – 29
• Glucose: 336
• BUN/Cr : 28/1.08
• Ca+ 9.7
• Vitamin B12, Folate – 1134 and 9.55
• Vitamin D – 20L
• Prolactin level - 10

10. LUMBAR PUNCTURE
• PROTEIN – 103
• GLUCOSE – 229
• COLORLESS
• WBC – 0
• RBC – 4
• VDRL – NON REACTIVE
• CSF GRAM STAIN AND CULTURE – NO GROWTH
URINE DRUG SCREEN IS POSITIVE FOR PRESCRIPTION OPIATES
MAYO CLINIC VIRAL ENCEPHALITIS PANEL – Positive for high VZV total Ab titres of 1:16 ref
is <1:2 but IgM titre is normal. LCM, Measles, Mumps, HSV, West nile were negative

11. • Imaging was done from the ER in the form of MRI of the Brain which is unremarkable.
• EEG was performed as inpatient which is reported as normal EEG.
IMAGING AND OTHER TESTS

12. CRANIAL NEURALGIAS SECONDARY TO ZOSTER
• Head or facial pain after Zoster is a well known entity but most commonly it affects
Trigeminal ganglion in 10-15% of cases and among them 80% of the lesions are confined
to the Ophthalmic branch. But it can affect geniculate ganglion causing eruptions in the
external auditory meatus, soft palate and upper cervical roots. It occurs in 10% of
Lymphoma and 25% of Hodgkin’s disease (ICHD2)
• Diagnostic criteria: (A+/-B+C+D)
A. Head or face pain in the distribution of a nerve or nerve division
B. Herpetic eruption in the same nerve territory
C. Pain precedes eruptions by <7days
D. Pain resolves in 3 months
POST –HERPETIC NEURALGIA:
Pain persisting or recurring after 3 months of onset of Herpes zoster. Sequel of zoster as the
age advances, affecting 50% of patients over the age of 60 years. Hyperesthesia,
hyperalgesia or allodynia are usually present in the territory involved.

13. OCCIPITAL NERVES AND THEIR DISTRIBUTION

14. • Greater occipital nerve – Dorsal primary ramus of C2. arised b/n the 1st and 2nd Cervical
vertebra along with lesser Occipital nerve. Ascends after emerging from the suboccipital
triangle beneath the obliquus capitus inferior muscle and then passes through the
Trapezius muscle and ascends to innervate the skin of the posterior scalp and vertex

15. • Lesser occipital nerve: Dorsal ramus of C2 and C3 nerves – innervates lateral area of the
head and posterior to the ear.
• CAUSES OF OCCIPITAL NEURALGIA

16. • The main symptom of this condition is chronic headache. The pain is commonly localized in the back
and around or over the top of the head, sometimes up to the eyebrow or behind the eye. Because
chronic headaches are a common symptom of numerous conditions, occipital neuralgia is often
misdiagnosed at first, most commonly as tension headache or a migraine leading to unsuccessful
treatment attempts. Another symptom is the eyes being sensitive to light, especially when headaches
occur.
• Occipital neuralgia is characterized by severe pain that begins in the upper neck and back of the head. This pain is
typically one-sided, although it can be on both sides if both occipital nerves have been affected. Additionally, the pain
may radiate forward toward the eye, as it follows the path of the occipital nerve(s). Individuals may notice blurred vision
as the pain radiates near or behind the eye. The neuralgia pain is commonly described as sharp, shooting, zapping, an
electric shock, or stabbing. The bouts of pain are rarely consistent, but can occur frequently with some patients
depending on the damage to the nerves. The amount of time the pain lasts typically varies each time the symptom
appears; it may last a few seconds or be almost continuous. Occipital neuralgia can last for hours or for several days.
Other symptoms of occipital neuralgia may include:
• Aching, burning, and throbbing pain that typically starts at the base of the head and radiates to the scalp
• Pain on one or both sides of the head
• Pain behind the eye
• Sensitivity to light
• Sensitivity to sound
• Slurred Speech
• Pain when moving the neck
• Difficulty with Balance and Coordination
• Tender scalp
• Nausea and/or vomiting

17. TREATMENT
• There are a wide range of non-invasive treatments, including alternative treatments,
which are as follows: acupuncture, chiropractic manipulation, occupational therapy,
osteopathic manipulation, massage, yoga, physical therapy, rest, heat, anti- inflammatory
medication, antidepressant medication, anti- convulsant medication, opioid and no opioid
analgesia, and migraine prophylaxis medication. Alternatives to these may include
local nerve block, peripheral nerve stimulation, steroids,
rhizotomy, phenol injections, antidepressants, and Occipital Cryoneurolysis.
• Other less common forms of surgical neurolysis or micro decompression are also used to
treat the condition when conservative measures fail.

18. MY SINCERE THANKS TO Dr. GOVINDARAJAN