Facial nerve paralysis as initial manifestation of amyloid beta related angiitis of CNS.

K. Fedorov MDa, T. Sergienko MDb, S.Golubev c E. Atyasovad, N. Nazarova MDb, P. Novikov MDa, S. Moiseev MDa

1. Clinic of Nephrology, Internal and Occupational Disease, First Moscow State Medical University, Moscow, Russia
2. First Clinical Hospital, Moscow, Russia
3. Moscow International Patomorphology Laboratory «LABORATOIRES DE GENIE».
4. International medical center “Petrovkamed”, Moscow, Russia

Objective

To our knowledge facial nerve paralysis is a rare manifestation of primary angiitis of nervous system (PACNS) including amyloid-beta related angiitis (ABRA).

Methods

Description of the clinical, radiological and histological findings.

Results

A 28 years old male developed peripheral right facial nerve paralysis in 2011, brain MRI was negative, the patient recovered completely in three weeks. A diffuse headache, diplopia and gate disturbance appeared soon after a mild closed head injury the same year. MRI showed multiple subcortical small hemorrhages in the right temporal lobe and in the pontine area. Brain noncontract MRA revealed no changes.  He worsened with dizziness, somnolence, vomiting and low-grade-fever in 2012, 2-fold CRP elevation was found. MRI showed progression of the cerebral small multiple hemorrhages and ischemic infarcts. Infectious, inherited, demyelinating, connective tissue diseases, antiphospholipid, paraneoplastic syndromes and coagulopathy were excluded, CSF was normal, low-grade fever and 2-3-fold CRP persisted. 16 mg of dexamethasone per day improved all the symptoms but was tapered off in two months. Along 2013-2014 the patient had several recurrent episodes of deterioration after a long alcohol abuse and another episode of closed head injury followed by multiplying of the hemorrhagic and ischemic brain lesions. A large temporal intracerebral hemorrhage (Picture 1) occurred in the beginning of the 2014 while taking hot bath; transtentorial herniation required a surgical brain decompression. Brain and meninges biopsy revealed granulomatous destructive angiitis with diffuse and local vessel beta-amyloid deposits in the meninges and brain tissue.  ABRA was diagnosed and treatment with three PRED 1500 mg IV pulses followed by 75 mg  orally  and  CYC 1400 mg IV every four weeks began. In two weeks temperature and CPR level came to normal, MRI showed no new lesions in two months.

Discussion

Facial nerve paralysis is a rare form of the disease onset. Granulomatous destructive angiitis accompanied by amyloid deposits, presence of multiple hemorrhagic and ischemic strokes, cognitive impairment, epilepsy in middle age are the main features of the disease.

Conclusion

ABRA is a potentially well treatable condition and is more susceptible to treatment with glucocorticosteroids and cytostatic agents then other forms of PACNS. Performing biopsy is crucial either in diagnosing of PACNS or deciding on antithrombotic treatment since antiplatelet and anticoagulant agents are undesirable in patients with amyloid angiopathy due to a high risk of hemorrhagic strokes.