Basal ganglion disease

Clinic

• Basal ganglia disease is a group of physical problems that occur when basal ganglia have problem.
• Reasons for abnormal increases or decreases of basal ganglia output are not yet well understood. But roughly, it can be said there are three form of basal Ganglia diseases:

1. Hyperkinetic disorders: Failing to properly suppress unwanted movements
2. Hypokinetic disorders: Failing to properly prime upper motor neuron circuits to initiate motor function.
3. Undefined form

(1) Hypokinetic disorders

• Parkinsonism: Rigidity, Rest tremor , and Slowness in initiation and execution of movement

(2) Hyperkinetic disorders

• Huntington's disease
• Dystonia: Characterized by involuntary movement and the slowing of intentional movement. Dystonia can occur as a hyperkinetic disorder or as a side effect of hypokinetic disorders such as Parkinson's disease.
• Hemiballismus: It is a hyperkinetic movement disorder that causes uncontrolled movement on one side of the body.

(3) Undefined form

The following diseases that generally involve the basal ganglia do not clearly fit into being either hypo- or hyperkinetic.

• Epilepsy

• Tourette syndrome / Obsessive–compulsive disorder: Tourette syndrome is a disorder that is characterized by behavioral and motor tics, OCD and ADHD. For this reason, it is commonly believed that pathologies involving limbic, associative and motor circuits of the basal ganglia are likely. Since the realization that syndromes such as Tourette syndrome and OCD are caused by dysfunction of the non-motor loops of basal ganglia circuits, new treatments for these disorders, based on treatments originally designed to treat movement disorders are being developed.

• Sydenham's chorea: Characterized by rapid, uncoordinated jerking movements primarily affecting Face / Hands / Feet. It is a result of an autoimmune response that occurs following infection by GABHS that destroys cells in the corpus striatum.

• PANDAS: PANDAS is a controversial hypothesis that there exists a subset of children with rapid onset of OCD or tic disorders and that these symptoms are caused by GABHS infections. The proposed link between infection and these disorders is that an initial autoimmune reaction to a GABHS infection produces antibodies that interfere with basal ganglia function, causing symptom exacerbations. It has been proposed that this autoimmune response can result in a broad range of neuropsychiatric symptoms.

• Dyskinetic cerebral palsy: It is a type of cerebral palsy primarily associated with damage to the basal ganglia in the form of lesions that occur during brain development due to bilirubin encephalopathy and hypoxic-ischemic brain injury. Symptoms include slow, uncontrolled movements of the extremities and trunk and small, rapid, random and repetitive, uncontrolled movements known as chorea. Involuntary movements often increase during periods of emotional stress or excitement and disappear when the patient is sleeping or distracted.

• Athymhormic syndrome: It is a rare psychopathological and neurological syndrome characterized by extreme passivity, apathy, blunted affect, and a profound generalized loss of self-motivation. The syndrome is believed to be due to damage to areas of the basal ganglia or frontal cortex, specifically the striatum and globus pallidus, responsible for motivation and executive functions.

• Lesch–Nyhan syndrome: A rare X-linked recessive disorder

• Wilson's disease: An autosomal recessive genetic disorder
• Fahr's disease: A rare, genetically dominant
• Blepharospasm: It is any abnormal contraction or twitch of the eyelid. Blepharospasm may come from abnormal functioning of the brain's basal ganglia.

Entities / Miasms

• Hypokinetic: Bradykinesia, Rigidity, Tremor, Parkinsonism: WEE
• Hyperkinetic: ChoreAthetosis, Psychosis, Hypertension: JE

Notes

• Hyperkinetic disorders are seen in NMDR encephalitis and also is a kind of Basal Ganglion disorder. So I consider Psychosis, Hypertention and Aphasia for it.
• Hypokinetic disorder, as it said in Athymhormic syndrome, has a Motivation decreasing feature, which is different from Depression. Its quality is similar to Schizophrenia. I think it is catatonic character and not Depressive.