Sub acute combined degeneration (B12 deficency)

B12 deficiency
Sub acute combined degeneration

Case :66 yo female vegetarian with 6 month history off distal extremities parasthesia accompany by 4 month complaint of ataxia and memory loss.
HGB : 10.4 g/dL (11.2-15.2)
MCV : 117 Fl (78-100)
MCH : 41.7 pg(27.4-33.0)
Homocysteine total : 59 (4 to 14)
Methylmalonic Acid (pending)
RBC morphology : macrocyosis,Polychromasia,Anisocytosis
B12 : 190 pg/mL (211-911)
Folate : 20.6 ng/mL

Macrocytic anemi
Polymorph
Hypersegmented
neutrophiles

Clinical Manifestation :

Hematologic
Neurologic
Neuropathy is symmetrical and affects the legs more than the arms.
It begins with paresthesias and ataxia associated with loss of vibration and position sense, and can progress to severe weakness, spasticity, clonus, paraplegia
Psychiatric disturbances, and dementia
Ischemic stroke
Optic neuropathy

Spongy or "vacuolar" degeneration of the spinal cord white matter

Management
4 injections of cyanocobalamin 1000 µg intramuscularly over 2 weeks.
In patients with pernicious anemia, monthly intramuscular injections of 1000 µg of cyanocobalamin

Guillain-Barré syndrome

Guillain-Barré syndrome
G.B.S
Acute inflammatory demyelinating polyradiculoneuropathy
A.I.D.P


French army neurologists in 1916
G. Guillain and J.A. Barré

Albuminocytologic dissociation
Etiology
Cell-mediated autoimmune disease of the peripheral nerves.
Nature of the antigens in acute Inflammatory demyelinating polyradiculoneuropathy is not known.
Variants of Guillain-Barré syndrome










CSF.Tx
1-Acute inflammatory demyelinating polyneuropathy
Symptoms and exam
Tendon reflexes
Severity
Evolution (1)
Autonomic function. Sixty-five percent of cases in some series have had manifestations of dysautonomia Sinus tachycardia occurs in more than 50% of severe cases (1).
EMG,CSF:
1-Ropper et al 1991



2-Acute motor-sensory axonal neuropathy
History: since 1916
Clinical picrure
Distinction from A.I.D.P
3-Acute motor axonal neuropathy (Chinese paralytic syndrome)
Clinical picture without sensory loss

Distinction from A.I.D.P
Campylobacter infection
4-Fisher syndrome
Clinical picture:
Characterized by 3 findings?
4-Fisher syndrome
Clinical picture: ophthalmoplegia, ataxia, areflexia

Also characterized by association with anti-GQ1b antibody
5-Sensory Guillain-Barré syndrome
6-pharyngeal-cervical-brachial variant
Limb muscles are spared ,manifests with acute oropharyngeal, neck, and shoulder weakness
7-Pandysautonomia
Acute or sub acute sympathetic and parasympathetic autonomic dysfunction with relative or complete preservation of somatic motor and sensory functions.
CSF, EMG, Autonomic study
Ganglionic acetylcholine receptors antibodies

Epidemiology
AIDP all parts of the world and at all ages
AIDP incidence is 0.6 to 2.4 cases per 100,000 population per year
Antecedent events
More than 50% AIDP follow by an infection
Other associated with AIDP include vaccination, surgery, epidural anesthesia, thrombolytic agents, and heroin use.
Diagnostic workup
CSF About 1 week after the onset of symptoms, the protein levels rise, reaching a peak in 3 to 4 weeks
Anti-GQ1b antibody
EMG


EMG
Electrophysiologic studies reveal a predominance of demyelinating features such as multifocal conduction block, slowing of nerve conduction velocities with prolonged distal and F-wave latencies, and temporal dispersion .
Temporal dispersion
EMG
Conduction studies are frequently normal at the beginning.

What might be the first change in EMG ?
Management
Corticosteroids randomized controlled trials revealed no benefit from such treatment (Hughes 1991)
Intravenous immunoglobulin, Plasmapheresis
Prevention of complications, such as respiratory failure Physical therapy