Gastrostomy in patients with prion disease

Figures & data

TABLE 1. Clinical characteristics of the cases of V180I Creutzfeldt-Jakob disease receiving gastrostomy.

	Case 1	Case 2	Case 3
Clinical features
Age at onset	78	76	69
Age at death	81	Alive	Alive
Sex	Female	Female	Female
Family history	−	−	−
Past history (age)	Cerebral infarction (73) Pituitary gland adenoma operation (65)	N. P.	Femoral neck fracture operation (50)
Comorbidity at clinical onset	Hypertension, Hyperlipidemia	N. P.	N. P.
Initial symptom	Disorientation	Abnormal behavior	Speech disturbance
Major symptoms and signs	Dementia, abnormal behavior	Dementia, disorientation	Dementia, disorientation
Cerebral cortical dysfunction	+	+	+
Visual symptoms	−	−	−
Parkinsonism	+	+	+
Cerebellar symptoms	−	−	−
Myoclonus	+	+	+
Startle reaction	+	+	+
Pathological laughing	+	+	+
MRI study
T2 cortical swelling	+	+	+
DWI hyperintensity	+	+	+
EEG study
PSWCs	−	−	−
Slowing	+	+	+
Akinetic mutism (Onset, months after admission)	16 months	17 months	13 months
Complications before gastrostomy*	N. P.	once aspiration pneumonia, twice sepsis	twice aspiration pneumonia
Disease duration until gastrostomy	30 months	38 months	14 months
Post-operative complications and treatment (Total episodes; total duration)	Bronchitis; Transluminal administration of antibiotic (2; 14 days) No episode of the intravenous administration	Bronchitis, folliculitis, and phlegmon; Transluminal (13; 127 days) and intravenous (1; 5 days) administrations of antibiotic	Bronchitis and phlegmon; Transluminal administrations of antibiotic (6; 65 days)
Episodes of tube feeding discontinuation	None Tube feeding was continued until death	None	None
Cause of death	Respiratory failure	Alive	Alive
Total disease duration	33 months	> 77 months	> 49 months
PrP gene analysis
Codon 129 polymorphism	Met/Met	Met/Met	Met/Met
Codon 219 polymorphism	Glu/Glu	Glu/Glu	Glu/Glu

+, present; —, absent; MRI, magnetic resonance imaging; DWI, diffusion-weighted imaging; EEG, electroencephalogram; PSWCs, periodic sharp-wave complexes; PrP, prion protein, Met, methionine; Glu, glutamic acid.

N. P., not present, PrP, prion protein. *Duration between onset of akinetic mutism and gastrostomy

TABLE 2. Clinical course and treatment of patients with prion disease following the advent of akinetic mutism.

						Administration
Case No.	Diagnosis	Observation period after induction of tube-feeding (months)	Coexistent disease (Therapy)	Complication (No. of times)	Cause of death	Nutrition (Calories (kcal.) / day)	Intravenous (No. of times; total duration)	Transluminal (No. of times; total duration)
Present series
1	V180I genetic CJD (Definite)	3	Hypertension, Hyperlipidemia (Hypotensor, Hyperlipidemia medicine)	BC (2)	RF	Gastrostomy (800 – 1,000)	None	Ab (2; 14 days)
2	V180I genetic CJD (Probable)	39	Hypertension (Hypotensor)	BC (6), Fo (6), Ph (1)	Alive	Gastrostomy (600 – 1,000)	No CI , Ab (1; 5 days)	Ab (13; 127 days)
3	V180I genetic CJD (Probable)	35	None	BC (5), Ph (1)	Alive	Gastrostomy (1,000)	None	Ab (6; 65 days)
Our previous report
A	V180I gCJD (Definite)	21	Pemphigoid (Steroid)	BC (6), GE (1),	RF	Nasal-tube (800)	CI (4; 42 days)(including CVC (1; 6 days); No HA), Ab (5; 20 days)	Ab (4; 26 days)
B	sCJD (Definite)	1	Bronchial asthma (Bronchodilator)	AP (1), BC (1),	RF	Nasal-tube (800)	CI (1; 18 days)(including CVC (1; 16 days); HA (1; 11 days)), Ab (1; 16 days)	Ab (1; 4 days)
C	sCJD (Definite)	23.5	None	AP (2), BC (15), HZ (1), UTI (1)	RF	Nasal-tube (800 – 1,000)	CI (2; 45 days)(including CVC (2; 35 days); HA (2; 29 days)), Ab (3; 29 days)	Ab (24; 94 days), Av (1; 7 days)
D	P102L GSS (Definite)	17.5	None	AP (3), BC (2), Cellulitis (2), PM (1)	RF with AP	Nasal-tube (600 – 800)	CI (3; 49 days)(including CVC (3; 60 days); HA (3; 35 days)), Ab (4; 51 days), Af (1; 11 days)	Ab (3; 23 days)
E	sCJD (Definite)	7	None	BC (6), UTI (1),	RF	Nasal-tube (1,000 – 1,400)	None	Ab (7; 55 days)
F	sCJD (Definite)	22	None	BC (9), GE (1)	RF	Nasal-tube (1,000)	None	Ab (10; 74 days)
G	sCJD (Definite)	8	None	AP (1), ARF (1), GE (7), UTI (1)	ARF with AP	Nasal-tube (800 – 1000)	CI (5; 124 days)(including CVC (2; 4 days); HA (2; 58 days)), Ab (3; 33 days)	Ab (3; 21 days)
H	sCJD (Probable)	8	None	BC (2)	—	Nasal-tube (1,000)	None	Ab (2; 14 days)
I	sCJD (Definite)	12	None	AP (1), BC (1)	RF with AP	Nasal-tube (1,000 kcal)	CI (1; 1 day), Ab (1; 1 day)	Ab (1; 7 days)
J	sCJD (Definite)	3.5	None	AP(1), BC (1), UTI (1), GE (2)	RF with AP	Nasal-tube (1,200)	CI (1; 5 days)(including CVC (1; 3 days); HA (1; 2 days)), Ab (1; 5 days)	Ab (3; 18 days)
K	sCJD (Probable)	8	Hypertension (Hypotensor)	GE (2)	—	Nasal-tube (1,000)	CI (1; 10 days), Ab (1; 10 days)	Ab (1; 3 days)
L	sCJD (Probable)	2	None	BC (2)	—	Nasal-tube (1,000)	CI(1; 14 days), Ab (1; 14 days)	Ab (1; 5 days)

CVC, central venous catheter; Ab, antibiotic; Af, antifungal; Av, antiviral.

AP, aspiration pneumonia; ARF, acute renal failure; BC, bronchitis; Fo, folliculitis; GE, gastroenteritis; HZ, herpes zoster; Ph, phlegmon; PM, pulmonary mycosis; RF, respiratory failure; UTI, urinary tract infection.

CI, continuous infusions, HA, hyperalimentation.

No. of times, Number of times that the treatment was administered; Total duration, the total length of time for which all treatments of the same type were administered.

Complication, Complication for which treatment was needed.

* Modified from our previous study.⁴

TABLE 3. Comparison of the nasal tube feeding group^* and gastric fistula tube feeding group.

		Proportion of the total observation period
(1) Total enforcement time (days) of continuous infusions
Nasal tube feeding group^*	308	7.8 %
Gastric fistula tube feeding group	0	0.0 %
Statistical analysis	p = 0.04^**
(2) Total days of drip infusion administration of antibiotic drugs
Nasal tube feeding group^*	179	4.5 %
Gastric fistula tube feeding group	5	0.2 %
Statistical analysis	N.S. (p = 0.11)^**
(3) Total days of transluminal administration of antibiotic drugs
Nasal tube feeding group^*	344	8.7 %
Gastric fistula tube feeding group	206	8.8 %
Statistical analysis	N.S. (p = 0.77)^**

Total observation periods were 3,968 d in 12 cases with nasal tube feeding, and 2341 d in 3 cases with gastric fistula tube feeding.

* Modified from our previous study.⁴

** Mann-Whitney's U test, N.S.; Statistically not significant.