multiple cranial nerve palsies

CASE PRESENTATION

DR. ANVESH NARIMETI1st yr postgraduate

INTERNAL MEDICINEGANDHI HOSPITAL

IDENTIFICATION DATA

NAME-PVK PRASAD

AGE-49 Yrs

SEX-MALE

OCCUPATION-TEACHER

ADDRESS-GUNTUR

RELIGION-HINDU

CHIEF COMPLAINT

Fever -45 days back

Swelling of right side of face -40 days back

Loss of vision in rt eye 35 days back

HISTORY OF PRESENT ILLNESSPatient was apparently normal and doing all his work

normally before 45 days and developed one episode of fever

• sudden onset• high grade associated with chills and rigors • continuous fever • associated with generalised body pains• subsided with medication After 2 days of fever patient noticed swelling of right side

of cheek not associated with pain.Gradually in two days patient developed swelling in rt

eye,,, not associated with pain,,, associated with redness of eye,, and protrusion of rt eye

• Followed by that patient had difficulty in moving the eye,,, and decreased vision in the rt eye,,, and patient unable to open the eye lid and with in span of 5 days patient completely lost vision in the rt eye

• Patient developed deviation of angle of mouth towards left side and patient presented to gandhi hospital after 10 days of presenting symptom

• h/o of loss of vision in rt eye• h/o of decreased sensation on the fore head and malar

prominence• No h/o difficulty in mastication• h/o of drooling of saliva from rt side of mouth, and

unable to fill the air in rt side of mouth• Deviation of angle of mouth towards lt side• No h/o of difficulty in hearing• Able to perceieve all the smells• No h/o of nasal regurgitation• No h/o difficulty in swallowing

• No h/o weakness of limbs and trunk• No h/o paresthesias and numbness• No h/o of trauma• No h/o of LOC• No h/o of ear discharge,• No h/o of halitosis,• No h/o of ear pain• No h/o of seizures,• No h/o of Headache• No h/o of neck pain,• No h/o of Vomtings• No h/o of jaw claudication

PAST HISTORY

• h/o of diabetes from last 15 yrs on irregular medication

• h/o of hypertension from last 3 montsirregular treatment

• No h/o of TB ,CAD ,CVA,EPILEPSY ,THYROID DISORDERS

• Personal h/o-strict vegetarian,non smoker,notan alcoholic,no other addictions,sleep and bowel and bladder habits are normal

• Family history-born out of consanguinousmarriage 4th in birth order out of 5,,,family h/o of diabetes in father and 3 other siblings.,,noother significant history

• Drug h/o- occasionally using OHA and anti hypertensives,

SUMMARY• 49 yrs old patient,,,TYPE 2 DM and HTN on

irregular treatment,,with fever and followed with H/O Suggestive of right sided 2nd , 3rd , 4th

, and 6th ,,and ophthalmic and maxillary division of 5th nerve and 7th nerve involvement with proptosis of rt eye ball with painless loss of vision.

DIFFERENTIAL DIAGNOSIS

• Orbital apex syndrome

• Cavernous sinus thrombosis

• Superior orbital fissure syndrome

• Fungal infection in uncontrolled diabetes-Mucormycosis,aspergillosis

• Tolasa hunt syndrome

• Orbital tumours

• Isoloated sphenoid sinus tumours

GENERAL EXAMINATION

• Patient ill looking ,,,toxic and no pallor

• no icterus,

• no cyanosis ,

• no clubbing,

• no lymphadenopathy

• no pedal edema

RIGHT EYE

• Blackish discolouration of peri orbital area and lids

• Edema of both lids

• Conjuctival chemosis +

• Proptosis - non pulsatile, non compressible,nontender,not variying with change in position.

• Perception of light absent

• Cornea hazy

• Pupils completely blocked by exudates

LEFT EYE -NORMAL

• Ear examination is –normal

• Nose examination –black discolaration of rtside of nose,,, nasal cavity is normal

• Broadening of nasal bridge

• Oral cavity –normal

• Oro pharynx-normal

• Rt side maxillary sinus tenderness present

VITALS

• BLOOD PRESSURE – 150/90 mm of hg rt arm supine position

• PULSE RATE-90 bpm regular in rythem,normalvolume,,, thickened vessel wall, no radio radial delay ,no radio femoral delay, all peripheral pulses are equally felt.

• RESPIRATORY RATE-18 per min abdominothoracic

CENTRAL NERVOUS SYSTEM

• HIGHER MENTAL FUNCTIONS – patient is conscious, coherent,oriented with time place and person,,

• Speech-dysarthria +,

• Memory intact

Cranial nerves

• 1ST NERVE-normal• 2ND NERVE- rt side absent perception of light

Fundus- cannot be examined on rt side. left side proliferative diabetic retinopathy

• 3rd ,4th ,6th cranial nerve rt side total ophthalmoplegia,, direct and indirect pupilary reflex absent, left side direct reflex is present indirect pupilary reflex is absent

• 5th nerve decreased sensation in ophthalmic and maxillary division on rt side,over mandibular region sensation is good and mastication is good.cornealreflex couldn’t be examined.

RIGHT 7TH NERVE

Deviation of angle of mouth to left side

loss of naso labial fold

Loss of frowning

Unable to hold air

Taste sensation lost

• Left 7th nerve normal

• 8TH NERVE-Patient is not cooperating for tuning fork tests

• 9th , 10th , 11th ,& 12th , cranial nerves are normal

• Motor system normal

• Sensory system- pinprick sensation reduced upto mid leg. Vibration and joint sensation normal

• CEREBELLAR SYSTEM NORMAL

• Gait normal

• Autonomic nervous system normal

• No signs of meningeal irritation

• Cardiovascular system- normal

• Respiratory system -normal

• Gastrointestinal system- normal

INVESTIGATIONS• HB-9.1, PCV 28%,TLC-17,200,PLT-4.2• DC-N88 L8 M3 E1• PT-12.9 APTT-22.6• BL00D UREA-66• SERUM CREATININE-2.5• NA 127 K 4.2• SGOT-24,SGPT-44 • RBS-500• ALP-330,SERUM PROTIENS-6.7GM• ALBUMIN-3.0• SERUM BILURUBIN-0.4• HIV NR HBSAG NR• ECG –NORMAL• CXR PA VIEW NORMAL• ESR –INCREASED• Usg abdomen –normal• 2d echo -normal

• CT BRAIN-HYPODENSITY IN RT TEMPORAL REGION-? CEREBRITIS

MUCOSAL THICKENING OF B/L ETHMOID AND SPHENOIDAL SINUS

• CT PNS- ORBITAL CELULITIS,soft tissue attenuation in rt pre septal,extra coronal fat with soft tissue swelling in rt periorbital region s/o inflamatory changes

• Pan sinusitis with blockage of rt osteomeatalunit,mild dns to rt

• MRI BRAIN- Enhancing altered signal intensity foci in right nasal cavity with extensivemucosalthickening of all paranasal sinuses with extension of lesion into the sphenoid bone and destroying the greater wing of rt sphenoidwith edema of rttemporal lobe-s/o fungal sinusitis

• Proptosis of rt eye ball

• Edema in subcutaneous tissue of rt cheek

• Infarcts in b/l frontal parietal,left temporal lobe white matter ,corona radiata&centrum semiovale

• Patient was subjected to FESS and intra nasal sinus biopsy taken from rt maxillary sinus and sent for lab

• KOH PREPARATION –showing plenty of branching septate hyphae

• Calcoflour white mount shows broad widely branching sparesly septate hyphae,resmblingzygomycetes

• Fungal culture showing growth of rhizopusspecies

Final diagnosis

• Uncontrolled Type 2 DM

• Uncontrolled Hypertension

• Rhinocerebral mucormycosis

• Rt orbital cellulitis

• Multiple cranial nerve lesions(2,3,4,6,7,v1 v2)

• Proliferative Diabetic retinopathy

• Diabetic neuropathy

MUCORMYCOSIS• Mucormycosis represents a group of life-threatening infections

caused by fungi of the order Mucorales.• Rhizopus oryzae (in the family Mucoraceae) is by far the most

common cause of infection• These fungi cause infection primarily in patients with diabetes or

defects in phagocytic function (e.g., associated with neutropenia or glucocorticoid treatment).

• Patients with elevated levels of free iron, which supports fungal growth in serum and tissues, are likewise at increased risk for mucormycosis.

• In iron-overloaded patients with end-stage renal failure, treatment with deferoxamine predisposes to the development of rapidly fatal disseminated mucormycosis; this agent, an iron chelator for the human host, serves as a fungal siderophore, directly delivering iron to the Mucorales.

• Mucormycosis typically occurs in patients with diabetes mellitus, solid organ or hematopoietic stem cell transplantation (HSCT), prolonged neutropenia, or malignancy

Mucormycosis can be divided into at least six clinical categories based on clinical presentation and the involvement of a particular anatomic site

1. Rhinocerebral(most common in diabetics)2. Pulmonary(in patients undergoing HSCT)3. Cutaneous4. Gastrointestinal5. Disseminated6. Miscellaneous

RHINOCEREBRAL MUCORMYCOSIS• The initial symptoms of rhinocerebral mucormycosis are nonspecific

and include eye or facial pain and facial numbness followed by the onset of conjunctival suffusion, blurry vision, and soft tissue swelling. Fever may be absent in up to half of cases, while white blood cell counts are typically elevated as long as the patient has functioning bone marrow

• If untreated, infection usually spreads from the ethmoid sinus to the orbit, resulting in compromise of extraocular muscle function and proptosis, typically with chemosis. Onset of signs and symptoms in the contralateral eye, with resulting bilateral proptosis, chemosis, vision loss, and ophthalmoplegia, is ominous and suggests the development of cavernous sinus thrombosis

• Upon visual inspection, infected tissue may appear to be normal during the earliest stages of fungal spread and then progresses through an erythematous phase, with or without edema, before the onset of a violaceous appearance and finally the development of a black necrotic eschar.

DIAGNOSIS• Definitive diagnosis requires a positive culture from a

sterile site (e.g., a needle aspirate, a tissue biopsy specimen, or pleural fluid)

• Histopathologic evidence of invasive mucormycosis.• A probable diagnosis of mucormycosis can be established

by culture from a nonsterile site (e.g., sputum or bronchoalveolar lavage)

• Biopsy with histopathologic examination remains the most sensitive and specific modality for definitive diagnosis .

• Biopsy reveals characteristic wide (6- to 30-m), thick-walled, ribbon-like, aseptate hyphal elements that branch at right angles

TREATMENT• AmB deoxycholate -1 mg/kg per day

• LAmB (LAmB) - 5–10 mg/kg per day

• amphotericin B lipid complex (ABLC) -5-7.5mg/kg

• However, dose escalation of LAmB to 10 mg/kg per day for CNS mucormycosis may be considered in light of the limited penetration of polyenes into the brain

• ABLC dose escalation above 7.5 mg/kg per day is not advisable

• Echinocandin–lipid polyene combinations inprimarily diabetic patients with rhino-orbital-cerebral mucormycosis

TAKE HOME MESSAGE• Multiple cranial nerves palsy should not be attributed to spontaneous

microvascular events affecting more than one cranial nerve at a time. This

remarkable coincidence does occur, especially in diabetic patients, but the

diagnosis is made only in retrospect after all other diagnostic alternatives

have been exhausted

• Neuroimaging should focus on the cavernous sinus, superior orbital

fissure, and orbital apex, where all three ocular motor nerves are in close

proximity.

• In a diabetic or immunocompromised host, fungal infection (Aspergillus,

Mucorales, Cryptococcus) is a common cause of multiple nerve palsies.

• In a patient with systemic malignancy, carcinomatous meningitis is a likely

diagnosis

•THANK YOU