ACQUIRED METABOLIC

DISORDERS OF THE
NERVOUS SYSTEM
Marianne Sajulga-Macias, MD

Classification of the acquired metabolic disorders of the nervous system in

adults
Presenting as a syndrome of confusion, stupor or coma
ischemia-hypoxia
hypercapnia
hypoglycemia
hyperglycemia
hepatic failure
reye syndrome
azotemia
disturbance of sodium, water balance & osmolality
acidosis due to DM or renal failure
Hashimotos disease
myxedema
Presenting as a progressive extrapyramidal symptoms
acquired hepatocerebral degeneration
hyperbilirubinemia & kernicterus
hypoparathyroidism
Presenting as cerebellar ataxia
hypothyroidism
hyperthermia
celiac sprue disease
Presenting as psychosis or dementia
Cushing disease & steroid encephalopathy
hyperthyroid psychosis & hypothyroidism
hyperparathyroidism
pancreatic encephalopathy

ISCHEMIC HYPOXIC
ENCEPHALOPATHY

Basic disorder is a lack of O2 and of blood flow to the brain, the

result of failure of the heart and circulation or of the lungs and
respiration

Medical conditions that most often lead to it are:

Global reduction in cerebral blood flow

MI

Ventricular arrhythmia

Aortic dissection

External or internal blood loss

Septic/traumatic shock

Hypoxia from suffocation

Drowning

Strangulation

Aspiration of vomitus, food or blood

Compression of the trachea by a mass or hemorrhage

Tracheal obstruction by a foreign body

General anesthesia accident

ISCHEMIC HYPOXIC ENCEPHALOPATHY

Medical conditions that most often lead to it are:

Diseases that paralyze the respiratory muscles

GBS

ALS

Myasthenia gravis

Damages to the medulla and leads to failure of

breathing

CO poisoning

ISCHEMIC HYPOXIC ENCEPHALOPATHY

Physiology of ischemic and

hypoxic damage

Autoregulation

There is compensatory
dilatation of resistance vessels
in response to a reduction in
cerebral perfusion, which
maintains blood flow at a
constant rate

ISCHEMIC HYPOXIC ENCEPHALOPATHY

Transient ischemia incomplete infarctions in the

border zones between major cerebral arteries

Predominant anoxia neurons in portions of

hippocampus and deep folia of the cerebellum are
vulnerable

Severe anoxia and hypoxia generalized damage

of the cerebral cortex, deep nuclei and cerebellum

Nuclear structures of the brainstem and spinal cord

Resistant to anoxia and hypotension and stop

functioning only after the cortex has been damaged

ISCHEMIC HYPOXIC ENCEPHALOPATHY

Clinical features of anoxic encephalopathy

Mild degree of hypoxia without LOC

Inattentiveness

Poor judgment

Incoordination

Degree of hypoxia that at no time abolish

consciousness rarely, if ever, cause permanent
damage to the nervous system

Severe global ischemia with prolonged LOC

Recovery can be complete if breathing, airway

and circulation are restored within 3-5 minutes

Beyond 5 minutes, there is usually permanent

damage

BRAIN DEATH SYNDROME

Most severe degree of

hypoxia, usually
caused by circulatory
arrest

Complete
unawareness and
unresponsiveness with
abolition of all
brainstem reflexes

POSTHYPOXIC NEUROLOGIC
SYNDROMES
1.

Persistent coma or stupor

2.

Dementia with or without extrapyramidal signs

3.

Extrapyramidal syndrome with cognitive impairment

4.

Choreoathetosis

5.

Cerebellar ataxia

6.

Intention or action myoclonus

7.

An amnesic state

POSTHYPOXIC NEUROLOGIC
SYNDROMES
Two watershed syndromes
1.

Visual agnosias including Balint

syndrome (optic ataxia,
psychic paralysis of gaze,
simultagnosia) and cortical
blindness

2.

Proximal arm and shoulder

weakness sometimes
accompanied by hip weakness
(man in a barrel)

PROGNOSIS OF HYPOXIC
ISCHEMIC BRAIN INJURY

According to Levy et al:

13% - attained a state of independent function within one year

25% of patients on admission has absent pupillary light reflex

none of them regained independence

10% - on admission has PLR, eye movements, motor response

better prognosis

Booth & colleagues, 5 clinical signs at 1 day after cardiac arrest

predicted a poor neurologic outcome or death:

Absent corneal reflex

Absent PLR

No withdrawal to pain

Absence of any motor response

TREATMENT OF HYPOXIC
ISCHEMIC BRAIN INJURY

Clear airway

Cardiopulmonary resuscitation

Hypothermia

Hypothermia After Cardiac Arrest Study Group

Reduce the core temperature to 33C within 2H of the arrest

and sustained this level for 12 hours in the first trial and
between 32C and 34C for 24hours in the second study

Both trials demonstrated improved survival and better

cognitive outcome in survivors

Control seizure

Myoclonus

Clonazepam 8-12mg daily in divided doses

HYPOGLYCEMIC ENCEPHALOPATHY

Essential biochemical abnormality is critical lowering of the

blood glucose

Glucose ~ 30mg/dl confusional state and seizure

Glucose ~ 10mg/dl coma

Normal brain has a reserve of 1-2gm (30mmol/100gm of brain

tissue) in the form of glycogen

Most common cause:

Accidental or deliberate overdose of insulin or diabetic agent

Islet cell insulin secreting pancreatic tumor

Depletion of the liver glycogen

Glycogen storage disorders

HYPOGLYCEMIC ENCEPHALOPATHY

Glucose ~30mg/dl

Glucose < 10mg/dl

Nervousness, hunger, flushed facie, sweating,

headache, palpitation, trembling, anxiety
Deep coma, dilatation of pupils, shallow respiration,
slow pulse, hypotonia

EEG diffuse slowing in the theta or delta range

UREMIC ENCEPHALOPATHY

Clinical manifestation:

Apathy, fatigue, inattentiveness, irritabilty usually

the initial symptoms
Later, there is confusion, dysarthria, tremor and
asterixis

EEG : diffusely and irregularly slow and may

remain for several weeks
Improvement of symptoms may not be evident
for a day or after institution of dialysis

Classification of the acquired metabolic disorders of the nervous system in

adults
Presenting as a syndrome of confusion, stupor or coma
ischemia-hypoxia
hypercapnia
hypoglycemia
hyperglycemia
hepatic failure
reye syndrome
azotemia
disturbance of sodium, water balance & osmolality
acidosis due to DM or renal failure
Hashimotos disease
myxedema
Presenting as a progressive extrapyramidal symptoms
acquired hepatocerebral degeneration
hyperbilirubinemia & kernicterus
hypoparathyroidism
Presenting as cerebellar ataxia
hypothyroidism
hyperthermia
celiac sprue disease
Presenting as psychosis or dementia
Cushing disease & steroid encephalopathy
hyperthyroid psychosis & hypothyroidism
hyperparathyroidism
pancreatic encephalopathy

HEPATOCEREBRAL DEGENERATION

Patients who survive an episode of hepatic coma

Clinical features

Tremor of the head and arm

Asterixis

Grimacing

Choreic movements

Twitching of the limbs

Dysarthria

Impairment of intellectual function

imaging: gliosis of both cerebral hemisphere and the

lenticular nuclei