Skip to main content

Table 2 Clinical findings and treatment outcomes in eight cases of WD with osseomuscular type

From: Clinical features and outcome in patients with osseomuscular type of Wilson’s disease

No

K-F ring

Neurologic Examination

CP (g/L)

Involvement on brain MRI

Abdominal ultrasonography

Outcomes of Chelation Therapy (Degreea)

Strength

Tone

Reflex

1

+

UE & LE 5

UE ↑

(++)

<0.02

Basal ganglia

Hepatic diffuse disease

(9 months after treatment) Dystonia in the right hand partially relieved; blurred speech persist; walking better (2)

2

+

UE & LE 5

Normal

(++)

0.02

Basal ganglia and brain stem

Hepatic diffuse disease, splenomegaly

(3 months after the treatment) Pain relieved with improved speech (0)

3

+

UE & LE 5

Normal

(++)

<0.02

Normal

Hepatic diffuse disease, splenomegaly

(14 months after treatment) Pain relieved (0)

4

+

UE & LE 5

UE & LE ↑

(++)

0.07

N/A

Hepatic diffuse disease

(after 23 months of inconsecutive treatment) No obvious improvement (3)

5

N/A

UE & LE 5

UE ↑

UE & LE (+++)

0.03

Basal ganglia and brain stem

Hepatic diffuse disease, splenomegaly

N/A

6

+

UE & LE 5

UE & LE ↓

(++)

0.03

N/A

Normal

N/A

7

+

UE 5; pLE 4; dLE 4-

UE & LE ↓

Knee (+++)

0.02

N/A

Hepatic diffuse disease, splenomegaly

N/A

8

-

UE 4; LE 4-

LE ↑

Knee (+++)

0.05

Normal

Increased echogenicity

(6 months after treatment) Pain relieved with improved joint motion (2)

  1. UE upper extremity, LE lower extremity, dLE distal lower extremity, pLE proximal lower extremity, CP ceruloplasmin, N/A not available
  2. aBased on the published assessment scale for osseomuscular symptoms [7]