A case of Arnolds Neuralgia

Naresh Mullaguri MD

Resident Physician PGY2

Department of Neurology

University of Missouri

A CASE OF ARNOLD’S NEURALGIA

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.

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

MEDICATIONS

• Lisinopril

• Atorvastatin

• ClementPercocet 10/325 Q4H

• Lantus

• Baclofen

• Flexeril

• Gabapentin

• Aspirin

• Pantoprazole

• Valacyclovir

• Prednisone 50mg daily should be tapered in a week

• Amitriptyline

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

PHYSICAL EXAMINATIONVitals: 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.

NEUROLOGICAL EXAMHIGHER 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.

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

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

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

• 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

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.

OCCIPITAL NERVES AND THEIR DISTRIBUTION

• 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

• 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

• 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

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.

MY SINCERE THANKS TO Dr. GOVINDARAJAN