Table 1 Comparisons of ³¹P MRS parameters in the hippocampus and premotor and motor cortices of MMD patients (before and after one-month Mg supplementation period) and control family members.

From: Brain of miyoshi myopathy/dysferlinopathy patients presents with structural and metabolic anomalies

³¹P MRS (m.mean ± SE)		PCr/ATP	PCr/P_i	P_i/ATP	PME/ATP	PME/PCr	PDE/ATP	PDE/PCr	PME/PDE	[Mg²⁺] (mM)
Motor cortex	Patients prior (pp)	0.15	1.07	0.12	0.21	1.43	0.52	3.56	0.46	0.09
	Patients prior (pp)	± 0.02	± 0.20	± 0.01	± 0.03	± 0.17	± 0.03	± 0.34	± 0.07	± 0.00
	Patients after (pa)	0.24	1.81	0.14	0.16	0.69	0.70	2.88	0.23	0.17
	Patients after (pa)	± 0.02	± 0.10	± 0.01	± 0.02	± 0.10	± 0.03	± 0.13	± 0.04	± 0.01
	Family controls (fCON)	0.19	1.77	0.10	0.13	0.74	0.61	3.24	0.22	0.18
	Family controls (fCON)	± 0.01	± 0.31	± 0.01	± 0.02	± 0.14	± 0.01	± 0.22	± 0.03	± 0.00
	pp vs fCON p-value	0.034	0.015	0.088	0.012	0.001	0.061	0.502	0.008	< 0.001
	pa vs fCON p-value	0.078	0.919	0.139	0.318	0.780	0.061	0.502	0.863	0.516
	pp vs pa p-value	0.032	0.023	0.486	0.258	0.011	0.019	0.502	0.035	< 0.001
Premotor cortex	Patients prior (pp)	0.18	1.19	0.14	0.22	1.29	0.51	2.99	0.44	0.15
	Patients prior (pp)	± 0.01	± 0.11	± 0.01	± 0.02	± 0.11	± 0.04	± 0.23	± 0.05	± 0.00
	Patients after (pa)	0.20	1.62	0.12	0.14	0.75	0.55	2.81	0.26	0.22
	Patients after (pa)	± 0.01	± 0.24	± 0.01	± 0.02	± 0.10	± 0.03	± 0.08	± 0.03	± 0.01
	Family controls (fCON)	0.22	1.98	0.12	0.13	0.60	0.60	2.72	0.21	0.24
	Family controls (fCON)	± 0.00	± 0.21	± 0.01	± 0.01	± 0.07	± 0.02	± 0.12	± 0.02	± 0.00
	pp vs Fcon p-value	0.034	0.014	0.088	0.005	0.001	0.061	0.502	0.006	< 0.001
	pa vs fCON p-value	0.206	0.340	0.668	0.571	0.305	0.296	0.610	0.236	0.100
	pp vs pa p-value	0.257	0.225	0.630	0.017	0.011	0.378	0.604	0.034	< 0.001
Hippocampus	Patients prior (pp)	0.20	1.33	0.14	0.22	1.23	0.47	2.44	0.48	0.14
	Patients prior (pp)	± 0.02	± 0.13	± 0.00	± 0.02	± 0.12	± 0.02	± 0.22	± 0.05	± 0.00
	Patients after (pa)	0.27	2.60	0.10	0.12	0.46	0.56	2.10	0.22	0.23
	Patients after (pa)	± 0.03	± 0.17	± 0.01	± 0.00	± 0.05	± 0.02	± 0.24	± 0.01	± 0.00
	Family controls (fCON)	0.24	2.21	0.12	0.13	0.53	0.55	2.22	0.23	0.22
	Family controls (fCON)	± 0.02	± 0.21	± 0.01	± 0.01	± 0.06	± 0.03	± 0.18	± 0.02	± 0.00
	pp vs fCON p-value	0.034	0.014	0.088	0.005	0.001	0.067	0.502	0.006	< 0.001
	pa vs fCON p-value	0.462	0.244	0.194	0.469	0.454	0.818	0.723	0.550	0.196
	pp vs pa p-value	0.096	0.005	0.002	0.003	0.001	0.054	0.512	0.006	< 0.001

Table summarizes ³¹P MRS metabolite ratios (marginal means ± SE), and [Mg²⁺] (marginal means ± SE) from the hippocampus and premotor and motor cortices of MMD patients (n = 4) and control family members (n = 5). The effect of Mg supplementation was considered in MMD patients.
ATP, adenosine triphosphates; fCON, control family members; [Mg²⁺], intracellular concentration of Mg²⁺; MMD, Miyoshi myopathy/dysferlinopathy; n, number of examined subjects; pa, MMD patients after treatment; PCr, phosphocreatine; PDE, phosphodiesters; P_i, inorganic phosphate; PME, phosphomonoesters; pp, MMD patients prior treatment.

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Table 1 Comparisons of ³¹P MRS parameters in the hippocampus and premotor and motor cortices of MMD patients (before and after one-month Mg supplementation period) and control family members.

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